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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2018-05-20 20:40:49 UTC
HMDB IDHMDB0000159
Secondary Accession Numbers
  • HMDB0000612
  • HMDB00159
  • HMDB00612
Metabolite Identification
Common NameL-Phenylalanine
DescriptionPhenylalanine is an essential amino acid and the precursor of the amino acid tyrosine. Like tyrosine, phenylalanine is also a precursor for catecholamines including tyramine, dopamine, epinephrine, and norepinephrine. Catecholamines are neurotransmitters that act as adrenalin-like substances. Interestingly, several psychotropic drugs (mescaline, morphine, codeine, and papaverine) also have phenylalanine as a constituent. Phenylalanine is highly concentrated in the human brain and plasma. Normal metabolism of phenylalanine requires biopterin, iron, niacin, vitamin B6, copper, and vitamin C. An average adult ingests 5 g of phenylalanine per day and may optimally need up to 8 g daily. Phenylalanine is highly concentrated in a number of high protein foods, such as meat, cottage cheese, and wheat germ. An additional dietary source of phenylalanine is artificial sweeteners containing aspartame. As a general rule, aspartame should be avoided by phenylketonurics and pregnant women. When present in sufficiently high levels, phenylalanine can act as a neurotoxin and a metabotoxin. A neurotoxin is a compound that disrupts or attacks neural cells and neural tissue. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of phenylalanine are associated with at least five inborn errors of metabolism, including Hartnup disorder, hyperphenylalaninemia due to guanosine triphosphate cyclohydrolase deficiency, phenylketonuria (PKU), tyrosinemia type 2 (or Richner-Hanhart syndrome), and tyrosinemia type III (TYRO3). Phenylketonurics have elevated serum plasma levels of phenylalanine up to 400 times normal. High plasma concentrations of phenylalanine influence the blood-brain barrier transport of large neutral amino acids. The high plasma phenylalanine concentrations increase phenylalanine entry into the brain and restrict the entry of other large neutral amino acids (PMID: 19191004 ). Phenylalanine has been found to interfere with different cerebral enzyme systems. Untreated phenylketonuria (PKU) can lead to intellectual disability, seizures, behavioural problems, and mental disorders. It may also result in a musty smell and lighter skin. Classic PKU dramatically affects myelination and white matter tracts in untreated infants; this may be one major cause of neurological disorders associated with phenylketonuria. Mild phenylketonuria can act as an unsuspected cause of hyperactivity, learning problems, and other developmental problems in children. It has been recently suggested that PKU may resemble amyloid diseases, such as Alzheimer's disease and Parkinson's disease, due to the formation of toxic amyloid-like assemblies of phenylalanine (PMID: 22706200 ). Phenylalanine also has some potential benefits. Phenylalanine can act as an effective pain reliever. Its use in premenstrual syndrome and Parkinson's may enhance the effects of acupuncture and electric transcutaneous nerve stimulation (TENS). Phenylalanine and tyrosine, like L-DOPA, produce a catecholamine-like effect. Phenylalanine is better absorbed than tyrosine and may cause fewer headaches. Low phenylalanine diets have been prescribed for certain cancers with mixed results. For instance, some tumours use more phenylalanine than others (particularly melatonin-producing tumours called melanomas).
Structure
Thumb
Synonyms
ValueSource
(S)-2-amino-3-Phenylpropionic acidChEBI
(S)-alpha-amino-beta-Phenylpropionic acidChEBI
3-Phenyl-L-alanineChEBI
beta-Phenyl-L-alanineChEBI
FChEBI
PheChEBI
PHENYLALANINEChEBI
(S)-2-amino-3-PhenylpropionateGenerator
(S)-a-amino-b-PhenylpropionateGenerator
(S)-a-amino-b-Phenylpropionic acidGenerator
(S)-alpha-amino-beta-PhenylpropionateGenerator
(S)-α-amino-β-phenylpropionateGenerator
(S)-α-amino-β-phenylpropionic acidGenerator
b-Phenyl-L-alanineGenerator
β-phenyl-L-alanineGenerator
(-)-beta-PhenylalanineHMDB
(L)-PhenylalanineHMDB
(S)-(-)-PhenylalanineHMDB
(S)-2-amino-3-PhenylpropanoateHMDB
(S)-2-amino-3-Phenylpropanoic acidHMDB
(S)-alpha-amino-BenzenepropanoateHMDB
(S)-alpha-amino-Benzenepropanoic acidHMDB
(S)-alpha-AminobenzenepropanoateHMDB
(S)-alpha-Aminobenzenepropanoic acidHMDB
(S)-alpha-AminohydrocinnamateHMDB
(S)-alpha-Aminohydrocinnamic acidHMDB
(S)-PhenylalanineHMDB
alpha-AminohydrocinnamateHMDB
alpha-Aminohydrocinnamic acidHMDB
beta-Phenyl-alpha-alanineHMDB
beta-PhenylalanineHMDB
L-2-amino-3-PhenylpropionateHMDB
L-2-amino-3-Phenylpropionic acidHMDB
Phenyl-alanineHMDB
PhenylalamineHMDB
L-Isomer phenylalanineMeSH
Phenylalanine, L isomerMeSH
Phenylalanine, L-isomerMeSH
EndorphenylMeSH
Chemical FormulaC9H11NO2
Average Molecular Weight165.1891
Monoisotopic Molecular Weight165.078978601
IUPAC Name(2S)-2-amino-3-phenylpropanoic acid
Traditional NameL-phenylalanine
CAS Registry Number63-91-2
SMILES
N[C@@H](CC1=CC=CC=C1)C(O)=O
InChI Identifier
InChI=1S/C9H11NO2/c10-8(9(11)12)6-7-4-2-1-3-5-7/h1-5,8H,6,10H2,(H,11,12)/t8-/m0/s1
InChI KeyCOLNVLDHVKWLRT-QMMMGPOBSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as phenylalanine and derivatives. These are compounds containing phenylalanine or a derivative thereof resulting from reaction of phenylalanine at the amino group or the carboxy group, or from the replacement of any hydrogen of glycine by a heteroatom.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassAmino acids, peptides, and analogues
Direct ParentPhenylalanine and derivatives
Alternative Parents
Substituents
  • Phenylalanine or derivatives
  • 3-phenylpropanoic-acid
  • Alpha-amino acid
  • Amphetamine or derivatives
  • L-alpha-amino acid
  • Aralkylamine
  • Monocyclic benzene moiety
  • Benzenoid
  • Amino acid
  • Carboxylic acid
  • Monocarboxylic acid or derivatives
  • Organic nitrogen compound
  • Primary amine
  • Organooxygen compound
  • Organonitrogen compound
  • Hydrocarbon derivative
  • Primary aliphatic amine
  • Organic oxide
  • Carbonyl group
  • Organopnictogen compound
  • Organic oxygen compound
  • Amine
  • Aromatic homomonocyclic compound
Molecular FrameworkAromatic homomonocyclic compounds
External Descriptors
Ontology
Physiological effect

Health effect:

Disposition

Route of exposure:

Source:

Biological location:

Process

Naturally occurring process:

Role

Indirect biological role:

Industrial application:

Biological role:

Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point283 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility26.9 mg/mLNot Available
LogP-1.38AVDEEF,A (1997)
Predicted Properties
PropertyValueSource
Water Solubility4.14 g/LALOGPS
logP-1.4ALOGPS
logP-1.2ChemAxon
logS-1.6ALOGPS
pKa (Strongest Acidic)2.47ChemAxon
pKa (Strongest Basic)9.45ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area63.32 ŲChemAxon
Rotatable Bond Count3ChemAxon
Refractivity45.12 m³·mol⁻¹ChemAxon
Polarizability17.03 ųChemAxon
Number of Rings1ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)splash10-0fr6-1930000000-a37fbccaf826443ef70cView in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)splash10-014l-1940000000-537e2725d621246630c1View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)splash10-00di-9630000000-ead0919f9a19d2352d80View in MoNA
GC-MSGC-MS Spectrum - GC-MS (1 TMS)splash10-00di-2900000000-cb2d6dc4bf9515150328View in MoNA
GC-MSGC-MS Spectrum - GC-MS (2 TMS)splash10-014l-2960000000-f77489792f0652dd5613View in MoNA
GC-MSGC-MS Spectrum - EI-B (Non-derivatized)splash10-014l-0970000000-792b341dd28b9e30bac2View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0fr6-1930000000-a37fbccaf826443ef70cView in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-014l-1940000000-537e2725d621246630c1View in MoNA
GC-MSGC-MS Spectrum - GC-EI-QQ (Non-derivatized)splash10-0ul9-3659000000-b85be4e71798e2fdc47bView in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-00di-9630000000-ead0919f9a19d2352d80View in MoNA
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-00di-2900000000-cb2d6dc4bf9515150328View in MoNA
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-014l-2960000000-f77489792f0652dd5613View in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-006x-9600000000-df38fcb743d8f44fb876View in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (1 TMS) - 70eV, Positivesplash10-00di-7900000000-f21569d2ec75b88e1bdaView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-014i-0900000000-0f3b994108b8a9fd2a56View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-0gb9-2900000000-c14d44c8a67621757f3dView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-004i-9300000000-08c642dab7f49c00da43View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-014i-0900000000-7dce1e473976f7d2143eView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-0f6x-9600000000-711557391093b0d8500aView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-00di-0900000000-0c25a5c116eac7bb059bView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-00di-0900000000-2804f79084ac4e67e155View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-014i-0900000000-8a71bb1f8424064d7cafView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-0f6x-9800000000-e027ff6bb67ce55e80a5View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-014i-0900000000-9f6185e9c7d54189f369View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-00di-0900000000-df5f72fe2bba91742427View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-0ir3-0988735721-bac229222fe7b52812a8View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-0002-0900000000-453477dec847a3672ffeView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-0a4i-0010963000-079a66bf710f6778bcebView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-00di-0000009000-62dbe98de4ecde484fb3View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negativesplash10-03di-0900000000-80558c17dc1845663c85View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negativesplash10-0002-3900000000-e1ee31d41e48824e84b7View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negativesplash10-0f6x-9500000000-cc11290a37615f24e16eView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Negativesplash10-0006-9000000000-1a6020bce0e1a9a14832View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negativesplash10-0006-9000000000-38c044a112152626962eView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Positivesplash10-014i-0900000000-9b908abfcb63153d60b3View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Positivesplash10-00di-1900000000-3ba5964e151bb1d56188View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Positivesplash10-00di-2900000000-20a7d24da0281f5b3b78View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Positivesplash10-0udi-5900000000-1800642a835b49f3398aView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Positivesplash10-0fb9-9300000000-f262384c85fb843f8a11View in MoNA
MSMass Spectrum (Electron Ionization)splash10-00dl-9300000000-4782928378caea601f9bView in MoNA
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR13C NMR SpectrumNot AvailableView in JSpectraViewer
2D NMR[1H,1H] 2D NMR SpectrumNot AvailableView in JSpectraViewer
2D NMR[1H,13C] 2D NMR SpectrumNot AvailableView in JSpectraViewer
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Mitochondria
Biospecimen Locations
  • Blood
  • Breast Milk
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Saliva
  • Sweat
  • Urine
Tissue Locations
  • All Tissues
  • Prostate
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified78.1 +/- 20.5 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified48 (50-52) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified85.2 +/- 23 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified57.0 (48.0-66.0) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified16-71 uMNewborn (0-30 days old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified24-80 uMInfant (1 - 3 months old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified34-101 uMChildren (3 months - 6 years old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified39-76 uMChildren (6 - 18 years old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified80.0 +/- 20.0 uMNewborn (0-30 days old)Not SpecifiedNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified60.0 +/- 8.0 uMChildren (1-13 years old)MaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified65.0 +/- 9.0 uMAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified56.0 +/- 8.0 uMAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified<70 uMNewborn (0-30 days old)Not SpecifiedNormal details
BloodDetected and Quantified108 (96.80-124) uMNewborn (0-30 days old)Not Available
Normal
details
BloodDetected and Quantified93.6 (75.8-114) uMInfant (0-1 year old)Not Available
Normal
details
BloodDetected and Quantified70(10.98) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified60 +/- 7 uMAdult (>18 years old)MaleNormal details
BloodDetected and Quantified52 +/- 5 uMAdult (>18 years old)FemaleNormal details
BloodDetected and Quantified47 +/- 5 uMChildren (1 - 13 years old)BothNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified40.0-75.0 uMAdult (>18 years old)Both
Normal
details
BloodDetected and Quantified56 +/- 8 uMAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified60.0 +/- 7.75 uMAdult (>18 years old)BothNormal details
BloodDetected but not Quantified Adult (>18 years old)Both
Normal
details
BloodDetected and Quantified65 +/- 9 uMAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified81.9(70.6-94.3) uMChildren (1-13 uears old)Both
Normal
details
BloodDetected and Quantified<120 uMNewborn (0-30 days old)Not SpecifiedNormal details
BloodDetected and Quantified166.0 +/- 3.0 uMAdult (>18 years old)BothNormal details
Breast MilkDetected and Quantified13.1 +/- 3.9 uMAdult (>18 years old)Female
Normal
details
Cerebrospinal Fluid (CSF)Detected and Quantified10.4 (7.58-13.2) uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified15 +/- 13 uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified18.0 +/- 7.2 uMAdult (>18 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified12.5 +/- 2.9 uMAdult (>18 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified10.8 +/- 4.2 uMAdult (>18 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified12.5 +/- 2.6 uMAdult (>18 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified4.3 +/- 0.81 uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified6.5 +/- 1.2 uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
Cerebrospinal Fluid (CSF)Detected and Quantified4-20 uMChildren (0 - 10 years old)Both
Normal
    • BC Children's Hos...
details
Cerebrospinal Fluid (CSF)Detected and Quantified6.6-15 uMAdolescent (>11 years old)Both
Normal
    • BC Children's Hos...
details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected but not Quantified Children (1-13 years old)BothNormal details
FecesDetected but not Quantified Adult (>18 years old)FemaleNormal details
FecesDetected but not Quantified Adult (>18 years old)BothNormal details
FecesDetected but not Quantified Infant (0-1 year old)Both
Normal
details
FecesDetected but not Quantified Not SpecifiedBoth
Normal
details
FecesDetected and Quantified3450.591 (2159.142-3652.380) nmol/g wet fecesInfant (0-1 year old)Not Specified
Normal
details
FecesDetected and Quantified3813.811 (665.904-6961.718) nmol/g wet fecesInfant (0-1 year old)Not Specified
Normal
details
FecesDetected and Quantified6396.709 (3632.201-9161.218) nmol/g wet fecesInfant (0-1 year old)Not Specified
Normal
details
FecesDetected but not Quantified Children (6 - 18 years old)BothNormal details
FecesDetected but not Quantified Children (6 - 18 years old)Not SpecifiedNormal details
FecesDetected but not Quantified Not SpecifiedNot Specified
Normal
details
FecesDetected but not Quantified Adult (>18 years old)FemaleNormal details
FecesDetected but not Quantified Not SpecifiedNot Specified
Normal
details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected but not Quantified Adult (>18 years old)Not Specified
Normal
details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected and Quantified41.770 +/- 42.134 nmol/g wet fecesNot SpecifiedNot Specified
Normal
details
FecesDetected but not Quantified Infant (0-1 year old)Both
Normal
details
FecesDetected but not Quantified Adult (>18 years old)FemaleNormal details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected and Quantified253.0433 +/- 397.726 nmol/g wet fecesNot SpecifiedNot Specified
Normal
details
FecesDetected and Quantified670 +/- 410 nmol/g wet fecesAdult (>18 years old)Both
Normal
details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected and Quantified120 +/- 120 nmol/g wet fecesAdult (>18 years old)Both
Normal
details
FecesDetected but not Quantified Adult (>18 years old)BothNormal details
FecesDetected but not Quantified Not SpecifiedNot Specified
Normal
details
FecesDetected but not Quantified Adult (>18 years old)MaleNormal details
SalivaDetected and Quantified10.69 +/- 6.28 uMAdult (>18 years old)FemaleNormal details
SalivaDetected and Quantified5.55 +/- 4.30 uMAdult (>18 years old)Female
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified<1-68 uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified<1-54 uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified18 (<1-112) uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified8 (<1-54) uMAdult (>18 years old)Female
Normal
details
SalivaDetected and Quantified8.24 +/- 6.51 uMAdult (>18 years old)Female
Normal
    • Sugimoto et al. (...
details
SalivaDetected but not Quantified Adult (>18 years old)BothNormal details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)BothNormal details
SalivaDetected and Quantified10.8 +/- 3.3 uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified1-187 uMAdult (>18 years old)Male
normal
details
SalivaDetected and Quantified1-32 uMAdult (>18 years old)Male
normal
details
SalivaDetected and Quantified12.0 +/- 6.5 uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified7.1 +/- 6.4 uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified43.70 +/- 23.30 uMAdult (>18 years old)BothNormal
    • Zerihun T. Dame, ...
details
SalivaDetected and Quantified10.3 +/- 3.8 uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified>10 uMAdult (>18 years old)BothNormal details
SweatDetected and Quantified40-260 uMAdult (60 years old)Male
Normal
details
SweatDetected and Quantified< 10 uMAdult (40 years old)Male
Normal
details
SweatDetected but not Quantified Adult BothNormal details
UrineDetected and Quantified3.17 - 37.65 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified6.90 - 32.79 umol/mmol creatinineInfant (1 - 6 months old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified11.76 - 32.79 umol/mmol creatinineInfant (6 months - <1 year old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified10.51 - 36.41 umol/mmol creatinineChildren (1 - 2 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified6.90 - 24.19 umol/mmol creatinineChildren (2 - 4 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified4.41 - 30.30 umol/mmol creatinineChildren (4 - 13 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified0.79 - 21.71 umol/mmol creatinineAdolescent (13 - 21 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified0.79 - 21.71 umol/mmol creatinineAdult (>21 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified7.8 (5.0-11.3) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified5.9 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified14.389 +/- 18.635 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Mordechai, Hien, ...
details
UrineDetected and Quantified3.63-18.17 umol/mmol creatinineAdult (>18 years old)BothNormal
    • David F. Putnam C...
details
UrineDetected and Quantified2.6-4.4 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified6.4 (3.5-11.2) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected but not Quantified Adult (>18 years old)Both
Normal
details
UrineDetected and Quantified4.605 (1.645-7.566) umol/mmol creatinineAdult (>18 years old)BothNormal
    details
    UrineDetected and Quantified4.50 +/- 1.15 umol/mmol creatinineAdult (>18 years old)BothNormal details
    UrineDetected and Quantified5.9 (2.8-10.8) umol/mmol creatinineAdult (>18 years old)Both
    Normal
    details
    UrineDetected and Quantified0.2 (0.07-0.35) umol/mmol creatinineNewborn (0-30 days old)BothNormal
      • Geigy Scientific ...
      • West Cadwell, N.J...
      • Basel, Switzerlan...
    details
    UrineDetected and Quantified1.61 +/- 1.0 umol/mmol creatinineChildren (1-13 years old)FemaleNormal
      • Geigy Scientific ...
      • West Cadwell, N.J...
      • Basel, Switzerlan...
    details
    UrineDetected and Quantified4.5 +/- 1.87 umol/mmol creatinineAdult (>18 years old)MaleNormal
      • Geigy Scientific ...
      • West Cadwell, N.J...
      • Basel, Switzerlan...
    details
    UrineDetected and Quantified4.0 +/- 1.76 umol/mmol creatinineAdult (>18 years old)FemaleNormal
      • Geigy Scientific ...
      • West Cadwell, N.J...
      • Basel, Switzerlan...
    details
    UrineDetected and Quantified3.1-5.0 umol/mmol creatinineAdult (>18 years old)MaleNormal details
    UrineDetected and Quantified3.7 umol/mmol creatinineAdult (>18 years old)BothNormal details
    UrineDetected and Quantified23.59 +/- 16.02 umol/mmol creatinineInfant (0-1 year old)BothNormal details
    UrineDetected and Quantified15.2043 +/- 4.9763 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
    Normal
      • Mordechai, Hien, ...
    details
    Abnormal Concentrations
    BiospecimenStatusValueAgeSexConditionReferenceDetails
    BloodDetected and Quantified60.3 +/- 16.8 uMChildren (1-13 years old)Both
    Obesity
      • Metabolomics reve...
    details
    BloodDetected and Quantified66.5 +/- 14.6 uMChildren (1-13 years old)Both
    Obesity
      • Metabolomics reve...
    details
    BloodDetected and Quantified49.6 (47.1-52.1) uMAdult (>18 years old)Both
    Epilepsy
    details
    BloodDetected and Quantified53.1 (50.8-55.4) uMChildren (1-13 years old)Both
    Epilepsy
    details
    BloodDetected and Quantified75.0 (67.0-83.0) uMAdult (>18 years old)BothDengue fever details
    BloodDetected and Quantified101.0 (97.0-105.0) uMAdult (>18 years old)BothMyocardial infarction details
    BloodDetected and Quantified98.0 +/- 113.0 uMNewborn (0-30 days old)BothMaple syrup urine disease (MSUD) details
    BloodDetected and Quantified143.0 (128.0-158.0) uMAdult (>18 years old)Both
    Bacterial infections (assorted)
    details
    BloodDetected and Quantified171.0 (140.0-202.0) uMAdult (>18 years old)Both
    Viral infections (assorted)
    details
    BloodDetected and Quantified571.0 +/- 1023.0 uMNewborn (0-30 days old)BothPhenylketonuria details
    BloodDetected and Quantified44.8 +/- 21 uMAdult (>18 years old)BothHeart Transplant details
    BloodDetected and Quantified74.91 +/- 34.67 uMAdult (>18 years old)Female
    Pregnancy with fetuses with trisomy 18
    details
    BloodDetected and Quantified58.24 +/- 28.67 uMAdult (>18 years old)FemalePregnancy details
    BloodDetected and Quantified62.9 (12.1) uMAdult (>18 years old)FemaleEarly preeclampsia details
    BloodDetected and Quantified79.6 (42.7) uMAdult (>18 years old)FemalePregnancy details
    BloodDetected but not Quantified Adult (>18 years old)BothColorectal cancer details
    BloodDetected and Quantified360-1200 uMNewborn (0-30 days old)BothHyperphenylalaninemia due to 6-pyruvoyltetrahydropterin synthase deficiency (PTPS) details
    BloodDetected and Quantified44.46 (12.81) uMAdult (>18 years old)FemalePregnancy with fetus having congenital heart defect details
    BloodDetected and Quantified1210.734 uMInfant (0-1 year old)MalePhenylketonuria details
    BloodDetected and Quantified43.9 (15.78) uMAdult (>18 years old)FemalePregnancy details
    BloodDetected and Quantified1004.909-1210.734 uMChildren (1 - 13 years old)MalePhenylketonuria details
    BloodDetected and Quantified78.0 (45.9) uMAdult (>18 years old)FemaleLate-onset preeclampsia details
    BloodDetected and Quantified76.83(15.96) uMAdult (>18 years old)BothHeart failure with preserved ejection fraction details
    BloodDetected and Quantified80.9 (45.4) uMAdult (>18 years old)FemalePregnancy details
    BloodDetected and Quantified64 +/- 8 uMAdult (>18 years old)MaleSchizophrenia details
    BloodDetected and Quantified57 +/- 8 uMAdolescent (13-18 years old)FemaleSchizophrenia details
    BloodDetected and Quantified39.954 uMNewborn (0-30 days old)FemaleHyperphenylalaninemia due to pterin-4a-carbinolamine dehydratase details
    BloodDetected and Quantified85.5(75.5-99.5) uMChildren (1-13 uears old)Both
    Environmental enteric dysfunction
    details
    BloodDetected and Quantified71.77 +/- 9.21 uMElderly (>65 years old)BothAlzheimer's disease details
    BloodDetected but not Quantified Adult (>18 years old)Both
    Pancreatic ductal adenocarcinoma
    details
    BloodDetected and Quantified1000-2400 uMNewborn (0-30 days old)MaleHyperphenylalaninemia due to guanosine triphosphate cyclohydrolase deficiency details
    BloodDetected and Quantified55.4 (27.0) uMAdult (>18 years old)FemaleDown syndrome pregnancy details
    BloodDetected and Quantified46.2 (30.8) uMAdult (>18 years old)FemalePregnancy details
    Cerebrospinal Fluid (CSF)Detected and Quantified14.7 (11.6-17.8) uMAdult (>18 years old)BothHypothyroidism details
    Cerebrospinal Fluid (CSF)Detected and Quantified18.6 +/- 5.2 uMChildren (1-13 years old)Not Specified
    Leukemia
    details
    Cerebrospinal Fluid (CSF)Detected and Quantified16.4 +/- 6.3 uMChildren (1-13 years old)Not SpecifiedLeukemia details
    Cerebrospinal Fluid (CSF)Detected and Quantified14.7 +/- 3.1 uMAdult (>18 years old)Not SpecifiedHypothyroidism details
    Cerebrospinal Fluid (CSF)Detected and Quantified6.0 +/- 0.8 uMAdult (>18 years old)BothAlzheimer's disease details
    Cerebrospinal Fluid (CSF)Detected and Quantified14.7 +/- 3.1 uMAdult (>18 years old)BothHypothyroidism details
    Cerebrospinal Fluid (CSF)Detected and Quantified4.6 +/- 1.0 uMAdult (>18 years old)BothSchizophrenia details
    FecesDetected but not Quantified Adult (>18 years old)Both
    Colorectal cancer
    details
    FecesDetected but not Quantified Adult (>18 years old)BothColorectal Cancer details
    FecesDetected but not Quantified Children (1-13 years old)BothAutism details
    FecesDetected but not Quantified Adult (>18 years old)Both
    Colorectal cancer
    details
    FecesDetected but not Quantified Adult (>18 years old)Femaleankylosing spondylitis details
    FecesDetected but not Quantified Adult (>18 years old)Femalerheumatoid arthritis details
    FecesDetected but not Quantified Adult (>18 years old)BothIleal Crohn's disease details
    FecesDetected but not Quantified Children (6 - 18 years old)BothCrohns disease details
    FecesDetected but not Quantified Children (6 - 18 years old)BothUlcerative colitis details
    FecesDetected but not Quantified Children (6 - 18 years old)Not SpecifiedCrohns disease details
    FecesDetected but not Quantified Children (6 - 18 years old)Not SpecifiedUlcerative colitis details
    FecesDetected but not Quantified Children (6 - 18 years old)Not SpecifiedUnclassified IBD details
    FecesDetected but not Quantified Not SpecifiedNot Specified
    Cryptosporidium infection
    details
    FecesDetected but not Quantified Adult (>18 years old)FemaleMyalgic encephalomyelitis/chronic fatigue syndrome details
    FecesDetected but not Quantified Adult (>18 years old)Both
    Irritable bowel syndrome
    details
    FecesDetected but not Quantified Adult (>18 years old)Both
    Ulcerative colitis
    details
    FecesDetected but not Quantified Adult (>18 years old)Both
    Crohn disease
    details
    FecesDetected but not Quantified Adult (>18 years old)Both
    Colorectal cancer
    details
    FecesDetected but not Quantified Adult (>18 years old)Both
    Ulcerative colitis
    details
    FecesDetected but not Quantified Adult (>18 years old)BothColorectal cancer details
    FecesDetected but not Quantified Adult (>18 years old)Both
    Irritable Bowel Syndrome
    details
    FecesDetected but not Quantified Adult (>18 years old)BothCrohns disease details
    FecesDetected but not Quantified Adult (>18 years old)BothUlcerative colitis details
    FecesDetected but not Quantified Adult (>18 years old)BothColorectal cancer details
    FecesDetected but not Quantified Adult (>18 years old)Both
    Colorectal cancer
    details
    FecesDetected but not Quantified Adult (>18 years old)MaleGout details
    SalivaDetected but not Quantified Adult (>18 years old)BothOral cancer details
    SalivaDetected but not Quantified Adult (>18 years old)FemaleBreast cancer details
    SalivaDetected but not Quantified Adult (>18 years old)Not SpecifiedPancreatic cancer details
    SalivaDetected but not Quantified Adult (>18 years old)Not SpecifiedPeriodontal diseases details
    SalivaDetected and Quantified5.97 +/- 0.09 uMAdult (>18 years old)MaleAlzheimer's disease details
    SalivaDetected but not Quantified Adult (>18 years old)BothOral squamous cell carcinoma (OSCC) details
    SalivaDetected but not Quantified Adult (>18 years old)BothOral leukoplakia (OLK) details
    SalivaDetected and Quantified9.01 +/- 2.41 uMAdult (>18 years old)MaleFrontotemporal lobe dementia details
    SalivaDetected and Quantified8.55 +/- 1.22 uMAdult (>18 years old)BothLewy body disease details
    UrineDetected and Quantified17.479 +/- 13.832 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
    Eosinophilic esophagitis
      • Mordechai, Hien, ...
    details
    UrineDetected and Quantified253.238 umol/mmol creatinineChildren (1 - 13 years old)MalePhenylketonuria details
    UrineDetected and Quantified414.763 umol/mmol creatinineInfant (0-1 year old)MalePhenylketonuria details
    UrineDetected and Quantified1.37 +/- 0.18 umol/mmol creatinineAdult (>18 years old)BothAlzheimer's disease details
    UrineDetected and Quantified11.9892 +/- 6.1922 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
    Eosinophilic esophagitis
      • Mordechai, Hien, ...
    details
    UrineDetected and Quantified10.2356 +/- 6.7416 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
    Gastroesophageal reflux disease
      • Mordechai, Hien, ...
    details
    Associated Disorders and Diseases
    Disease References
    6-Pyruvoyltetrahydropterin synthase deficiency
    1. Thony B, Leimbacher W, Blau N, Harvie A, Heizmann CW: Hyperphenylalaninemia due to defects in tetrahydrobiopterin metabolism: molecular characterization of mutations in 6-pyruvoyl-tetrahydropterin synthase. Am J Hum Genet. 1994 May;54(5):782-92. [PubMed:8178819 ]
    Alzheimer's disease
    1. Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed:17031479 ]
    2. Tsuruoka M, Hara J, Hirayama A, Sugimoto M, Soga T, Shankle WR, Tomita M: Capillary electrophoresis-mass spectrometry-based metabolome analysis of serum and saliva from neurodegenerative dementia patients. Electrophoresis. 2013 Oct;34(19):2865-72. doi: 10.1002/elps.201300019. Epub 2013 Sep 6. [PubMed:23857558 ]
    Pregnancy
    1. Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: Metabolomics and first-trimester prediction of early-onset preeclampsia. J Matern Fetal Neonatal Med. 2012 Oct;25(10):1840-7. doi: 10.3109/14767058.2012.680254. Epub 2012 Apr 28. [PubMed:22494326 ]
    2. Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: First-trimester metabolomic detection of late-onset preeclampsia. Am J Obstet Gynecol. 2013 Jan;208(1):58.e1-7. doi: 10.1016/j.ajog.2012.11.003. Epub 2012 Nov 13. [PubMed:23159745 ]
    3. Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: Metabolomic analysis for first-trimester Down syndrome prediction. Am J Obstet Gynecol. 2013 May;208(5):371.e1-8. doi: 10.1016/j.ajog.2012.12.035. Epub 2013 Jan 8. [PubMed:23313728 ]
    4. Bahado-Singh RO, Akolekar R, Chelliah A, Mandal R, Dong E, Kruger M, Wishart DS, Nicolaides K: Metabolomic analysis for first-trimester trisomy 18 detection. Am J Obstet Gynecol. 2013 Jul;209(1):65.e1-9. doi: 10.1016/j.ajog.2013.03.028. Epub 2013 Mar 25. [PubMed:23535240 ]
    5. Bahado-Singh RO, Ertl R, Mandal R, Bjorndahl TC, Syngelaki A, Han B, Dong E, Liu PB, Alpay-Savasan Z, Wishart DS, Nicolaides KH: Metabolomic prediction of fetal congenital heart defect in the first trimester. Am J Obstet Gynecol. 2014 Sep;211(3):240.e1-240.e14. doi: 10.1016/j.ajog.2014.03.056. Epub 2014 Apr 1. [PubMed:24704061 ]
    Autism
    1. De Angelis M, Piccolo M, Vannini L, Siragusa S, De Giacomo A, Serrazzanetti DI, Cristofori F, Guerzoni ME, Gobbetti M, Francavilla R: Fecal microbiota and metabolome of children with autism and pervasive developmental disorder not otherwise specified. PLoS One. 2013 Oct 9;8(10):e76993. doi: 10.1371/journal.pone.0076993. eCollection 2013. [PubMed:24130822 ]
    Bacterial infections
    1. Wannemacher RW Jr, Klainer AS, Dinterman RE, Beisel WR: The significance and mechanism of an increased serum phenylalanine-tyrosine ratio during infection. Am J Clin Nutr. 1976 Sep;29(9):997-1006. [PubMed:822705 ]
    Perillyl alcohol administration for cancer treatment
    1. Sugimoto M, Wong DT, Hirayama A, Soga T, Tomita M: Capillary electrophoresis mass spectrometry-based saliva metabolomics identified oral, breast and pancreatic cancer-specific profiles. Metabolomics. 2010 Mar;6(1):78-95. Epub 2009 Sep 10. [PubMed:20300169 ]
    Colorectal cancer
    1. Weir TL, Manter DK, Sheflin AM, Barnett BA, Heuberger AL, Ryan EP: Stool microbiome and metabolome differences between colorectal cancer patients and healthy adults. PLoS One. 2013 Aug 6;8(8):e70803. doi: 10.1371/journal.pone.0070803. Print 2013. [PubMed:23940645 ]
    2. Ni Y, Xie G, Jia W: Metabonomics of human colorectal cancer: new approaches for early diagnosis and biomarker discovery. J Proteome Res. 2014 Sep 5;13(9):3857-70. doi: 10.1021/pr500443c. Epub 2014 Aug 14. [PubMed:25105552 ]
    3. Brown DG, Rao S, Weir TL, O'Malia J, Bazan M, Brown RJ, Ryan EP: Metabolomics and metabolic pathway networks from human colorectal cancers, adjacent mucosa, and stool. Cancer Metab. 2016 Jun 6;4:11. doi: 10.1186/s40170-016-0151-y. eCollection 2016. [PubMed:27275383 ]
    4. Sinha R, Ahn J, Sampson JN, Shi J, Yu G, Xiong X, Hayes RB, Goedert JJ: Fecal Microbiota, Fecal Metabolome, and Colorectal Cancer Interrelations. PLoS One. 2016 Mar 25;11(3):e0152126. doi: 10.1371/journal.pone.0152126. eCollection 2016. [PubMed:27015276 ]
    5. Goedert JJ, Sampson JN, Moore SC, Xiao Q, Xiong X, Hayes RB, Ahn J, Shi J, Sinha R: Fecal metabolomics: assay performance and association with colorectal cancer. Carcinogenesis. 2014 Sep;35(9):2089-96. doi: 10.1093/carcin/bgu131. Epub 2014 Jul 18. [PubMed:25037050 ]
    6. Wang X, Wang J, Rao B, Deng L: Gut flora profiling and fecal metabolite composition of colorectal cancer patients and healthy individuals. Exp Ther Med. 2017 Jun;13(6):2848-2854. doi: 10.3892/etm.2017.4367. Epub 2017 Apr 20. [PubMed:28587349 ]
    Crohn's disease
    1. Bjerrum JT, Wang Y, Hao F, Coskun M, Ludwig C, Gunther U, Nielsen OH: Metabonomics of human fecal extracts characterize ulcerative colitis, Crohn's disease and healthy individuals. Metabolomics. 2015;11:122-133. Epub 2014 Jun 1. [PubMed:25598765 ]
    2. Kolho KL, Pessia A, Jaakkola T, de Vos WM, Velagapudi V: Faecal and Serum Metabolomics in Paediatric Inflammatory Bowel Disease. J Crohns Colitis. 2017 Mar 1;11(3):321-334. doi: 10.1093/ecco-jcc/jjw158. [PubMed:27609529 ]
    3. Azario I, Pievani A, Del Priore F, Antolini L, Santi L, Corsi A, Cardinale L, Sawamoto K, Kubaski F, Gentner B, Bernardo ME, Valsecchi MG, Riminucci M, Tomatsu S, Aiuti A, Biondi A, Serafini M: Neonatal umbilical cord blood transplantation halts skeletal disease progression in the murine model of MPS-I. Sci Rep. 2017 Aug 25;7(1):9473. doi: 10.1038/s41598-017-09958-9. [PubMed:28842642 ]
    Dengue fever
    1. Klassen P, Furst P, Schulz C, Mazariegos M, Solomons NW: Plasma free amino acid concentrations in healthy Guatemalan adults and in patients with classic dengue. Am J Clin Nutr. 2001 Mar;73(3):647-52. [PubMed:11237944 ]
    Epilepsy
    1. Rainesalo S, Keranen T, Palmio J, Peltola J, Oja SS, Saransaari P: Plasma and cerebrospinal fluid amino acids in epileptic patients. Neurochem Res. 2004 Jan;29(1):319-24. [PubMed:14992292 ]
    Schizophrenia
    1. Do KQ, Lauer CJ, Schreiber W, Zollinger M, Gutteck-Amsler U, Cuenod M, Holsboer F: gamma-Glutamylglutamine and taurine concentrations are decreased in the cerebrospinal fluid of drug-naive patients with schizophrenic disorders. J Neurochem. 1995 Dec;65(6):2652-62. [PubMed:7595563 ]
    2. Bjerkenstedt L, Edman G, Hagenfeldt L, Sedvall G, Wiesel FA: Plasma amino acids in relation to cerebrospinal fluid monoamine metabolites in schizophrenic patients and healthy controls. Br J Psychiatry. 1985 Sep;147:276-82. [PubMed:2415198 ]
    Frontotemporal dementia
    1. Tsuruoka M, Hara J, Hirayama A, Sugimoto M, Soga T, Shankle WR, Tomita M: Capillary electrophoresis-mass spectrometry-based metabolome analysis of serum and saliva from neurodegenerative dementia patients. Electrophoresis. 2013 Oct;34(19):2865-72. doi: 10.1002/elps.201300019. Epub 2013 Sep 6. [PubMed:23857558 ]
    Guanosine triphosphate cyclohydrolase deficiency
    1. Dhondt JL, Farriaux JP, Boudha A, Largilliere C, Ringel J, Roger MM, Leeming RJ: Neonatal hyperphenylalaninemia presumably caused by guanosine triphosphate-cyclohydrolase deficiency. J Pediatr. 1985 Jun;106(6):954-6. [PubMed:3873535 ]
    Hypothyroidism
    1. Sjoberg S, Eriksson M, Nordin C: L-thyroxine treatment and neurotransmitter levels in the cerebrospinal fluid of hypothyroid patients: a pilot study. Eur J Endocrinol. 1998 Nov;139(5):493-7. [PubMed:9849813 ]
    Irritable bowel syndrome
    1. Le Gall G, Noor SO, Ridgway K, Scovell L, Jamieson C, Johnson IT, Colquhoun IJ, Kemsley EK, Narbad A: Metabolomics of fecal extracts detects altered metabolic activity of gut microbiota in ulcerative colitis and irritable bowel syndrome. J Proteome Res. 2011 Sep 2;10(9):4208-18. doi: 10.1021/pr2003598. Epub 2011 Aug 8. [PubMed:21761941 ]
    2. Hong YS, Hong KS, Park MH, Ahn YT, Lee JH, Huh CS, Lee J, Kim IK, Hwang GS, Kim JS: Metabonomic understanding of probiotic effects in humans with irritable bowel syndrome. J Clin Gastroenterol. 2011 May-Jun;45(5):415-25. doi: 10.1097/MCG.0b013e318207f76c. [PubMed:21494186 ]
    Leukemia
    1. Peng CT, Wu KH, Lan SJ, Tsai JJ, Tsai FJ, Tsai CH: Amino acid concentrations in cerebrospinal fluid in children with acute lymphoblastic leukemia undergoing chemotherapy. Eur J Cancer. 2005 May;41(8):1158-63. Epub 2005 Apr 14. [PubMed:15911239 ]
    Maple syrup urine disease
    1. Deng C, Shang C, Hu Y, Zhang X: Rapid diagnosis of phenylketonuria and other aminoacidemias by quantitative analysis of amino acids in neonatal blood spots by gas chromatography-mass spectrometry. J Chromatogr B Analyt Technol Biomed Life Sci. 2002 Jul 25;775(1):115-20. [PubMed:12101068 ]
    Myocardial infarction
    1. Wannemacher RW Jr, Klainer AS, Dinterman RE, Beisel WR: The significance and mechanism of an increased serum phenylalanine-tyrosine ratio during infection. Am J Clin Nutr. 1976 Sep;29(9):997-1006. [PubMed:822705 ]
    Obesity
    1. Simone Wahl, Christina Holzapfel, Zhonghao Yu, Michaela Breier, Ivan Kondofersky, Christiane Fuchs, Paula Singmann, Cornelia Prehn, Jerzy Adamski, Harald Grallert, Thomas Illig, Rui Wang-Sattler, Thomas Reinehr (2013). Metabolomics reveals determinants of weight loss during lifestyle intervention in obese children. Metabolomics.
    Periodontal disease
    1. Sugimoto M, Wong DT, Hirayama A, Soga T, Tomita M: Capillary electrophoresis mass spectrometry-based saliva metabolomics identified oral, breast and pancreatic cancer-specific profiles. Metabolomics. 2010 Mar;6(1):78-95. Epub 2009 Sep 10. [PubMed:20300169 ]
    Phenylketonuria
    1. Deng C, Shang C, Hu Y, Zhang X: Rapid diagnosis of phenylketonuria and other aminoacidemias by quantitative analysis of amino acids in neonatal blood spots by gas chromatography-mass spectrometry. J Chromatogr B Analyt Technol Biomed Life Sci. 2002 Jul 25;775(1):115-20. [PubMed:12101068 ]
    2. Swarna M, Jyothy A, Usha Rani P, Reddy PP: Amino acid disorders in mental retardation: a two-decade study from Andhra Pradesh. Biochem Genet. 2004 Apr;42(3-4):85-98. [PubMed:15168722 ]
    Pterin-4a carbinolamine dehydratase deficiency
    1. Blaskovics M, Giudici TA: A new variant of biopterin deficiency. N Engl J Med. 1988 Dec 15;319(24):1611-2. doi: 10.1056/NEJM198812153192420. [PubMed:3200274 ]
    Rheumatoid arthritis
    1. Tie-juan ShaoZhi-xing HeZhi-jun XieHai-chang LiMei-jiao WangCheng-ping Wen. Characterization of ankylosing spondylitis and rheumatoid arthritis using 1H NMR-based metabolomics of human fecal extracts. Metabolomics. April 2016, 12:70 [Link]
    Viral infection
    1. Wannemacher RW Jr, Klainer AS, Dinterman RE, Beisel WR: The significance and mechanism of an increased serum phenylalanine-tyrosine ratio during infection. Am J Clin Nutr. 1976 Sep;29(9):997-1006. [PubMed:822705 ]
    Pancreatic cancer
    1. Sugimoto M, Wong DT, Hirayama A, Soga T, Tomita M: Capillary electrophoresis mass spectrometry-based saliva metabolomics identified oral, breast and pancreatic cancer-specific profiles. Metabolomics. 2010 Mar;6(1):78-95. Epub 2009 Sep 10. [PubMed:20300169 ]
    Gout
    1. Shao T, Shao L, Li H, Xie Z, He Z, Wen C: Combined Signature of the Fecal Microbiome and Metabolome in Patients with Gout. Front Microbiol. 2017 Feb 21;8:268. doi: 10.3389/fmicb.2017.00268. eCollection 2017. [PubMed:28270806 ]
    Eosinophilic esophagitis
    1. (). Mordechai, Hien, and David S. Wishart. .
    Ulcerative colitis
    1. Le Gall G, Noor SO, Ridgway K, Scovell L, Jamieson C, Johnson IT, Colquhoun IJ, Kemsley EK, Narbad A: Metabolomics of fecal extracts detects altered metabolic activity of gut microbiota in ulcerative colitis and irritable bowel syndrome. J Proteome Res. 2011 Sep 2;10(9):4208-18. doi: 10.1021/pr2003598. Epub 2011 Aug 8. [PubMed:21761941 ]
    2. Bjerrum JT, Wang Y, Hao F, Coskun M, Ludwig C, Gunther U, Nielsen OH: Metabonomics of human fecal extracts characterize ulcerative colitis, Crohn's disease and healthy individuals. Metabolomics. 2015;11:122-133. Epub 2014 Jun 1. [PubMed:25598765 ]
    3. Kolho KL, Pessia A, Jaakkola T, de Vos WM, Velagapudi V: Faecal and Serum Metabolomics in Paediatric Inflammatory Bowel Disease. J Crohns Colitis. 2017 Mar 1;11(3):321-334. doi: 10.1093/ecco-jcc/jjw158. [PubMed:27609529 ]
    4. Azario I, Pievani A, Del Priore F, Antolini L, Santi L, Corsi A, Cardinale L, Sawamoto K, Kubaski F, Gentner B, Bernardo ME, Valsecchi MG, Riminucci M, Tomatsu S, Aiuti A, Biondi A, Serafini M: Neonatal umbilical cord blood transplantation halts skeletal disease progression in the murine model of MPS-I. Sci Rep. 2017 Aug 25;7(1):9473. doi: 10.1038/s41598-017-09958-9. [PubMed:28842642 ]
    Early preeclampsia
    1. Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: Metabolomics and first-trimester prediction of early-onset preeclampsia. J Matern Fetal Neonatal Med. 2012 Oct;25(10):1840-7. doi: 10.3109/14767058.2012.680254. Epub 2012 Apr 28. [PubMed:22494326 ]
    Late-onset preeclampsia
    1. Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: First-trimester metabolomic detection of late-onset preeclampsia. Am J Obstet Gynecol. 2013 Jan;208(1):58.e1-7. doi: 10.1016/j.ajog.2012.11.003. Epub 2012 Nov 13. [PubMed:23159745 ]
    Lewy body disease
    1. Tsuruoka M, Hara J, Hirayama A, Sugimoto M, Soga T, Shankle WR, Tomita M: Capillary electrophoresis-mass spectrometry-based metabolome analysis of serum and saliva from neurodegenerative dementia patients. Electrophoresis. 2013 Oct;34(19):2865-72. doi: 10.1002/elps.201300019. Epub 2013 Sep 6. [PubMed:23857558 ]
    Associated OMIM IDs
    DrugBank IDDB00120
    Phenol Explorer Compound IDNot Available
    FoodDB IDFDB014705
    KNApSAcK IDC00001386
    Chemspider ID5910
    KEGG Compound IDC00079
    BioCyc IDPHE
    BiGG ID33775
    Wikipedia LinkPhenylalanine
    METLIN ID28
    PubChem Compound6140
    PDB IDPHE
    ChEBI ID17295
    References
    Synthesis ReferenceZhou, Hua; Zhong, Yao; Sun, Guanghai; Wei, Ping. Preparation of L-phenylalanine by an aqueous two-phase system. Huaxue Fanying Gongcheng Yu Gongyi (2006), 22(2), 146-150.
    Material Safety Data Sheet (MSDS)Download (PDF)
    General References
    1. Sreekumar A, Poisson LM, Rajendiran TM, Khan AP, Cao Q, Yu J, Laxman B, Mehra R, Lonigro RJ, Li Y, Nyati MK, Ahsan A, Kalyana-Sundaram S, Han B, Cao X, Byun J, Omenn GS, Ghosh D, Pennathur S, Alexander DC, Berger A, Shuster JR, Wei JT, Varambally S, Beecher C, Chinnaiyan AM: Metabolomic profiles delineate potential role for sarcosine in prostate cancer progression. Nature. 2009 Feb 12;457(7231):910-4. doi: 10.1038/nature07762. [PubMed:19212411 ]
    2. Silwood CJ, Lynch E, Claxson AW, Grootveld MC: 1H and (13)C NMR spectroscopic analysis of human saliva. J Dent Res. 2002 Jun;81(6):422-7. [PubMed:12097436 ]
    3. Nicholson JK, O'Flynn MP, Sadler PJ, Macleod AF, Juul SM, Sonksen PH: Proton-nuclear-magnetic-resonance studies of serum, plasma and urine from fasting normal and diabetic subjects. Biochem J. 1984 Jan 15;217(2):365-75. [PubMed:6696735 ]
    4. Engelborghs S, Marescau B, De Deyn PP: Amino acids and biogenic amines in cerebrospinal fluid of patients with Parkinson's disease. Neurochem Res. 2003 Aug;28(8):1145-50. [PubMed:12834252 ]
    5. Sjoberg S, Eriksson M, Nordin C: L-thyroxine treatment and neurotransmitter levels in the cerebrospinal fluid of hypothyroid patients: a pilot study. Eur J Endocrinol. 1998 Nov;139(5):493-7. [PubMed:9849813 ]
    6. Hagenfeldt L, Bjerkenstedt L, Edman G, Sedvall G, Wiesel FA: Amino acids in plasma and CSF and monoamine metabolites in CSF: interrelationship in healthy subjects. J Neurochem. 1984 Mar;42(3):833-7. [PubMed:6198473 ]
    7. Peng CT, Wu KH, Lan SJ, Tsai JJ, Tsai FJ, Tsai CH: Amino acid concentrations in cerebrospinal fluid in children with acute lymphoblastic leukemia undergoing chemotherapy. Eur J Cancer. 2005 May;41(8):1158-63. Epub 2005 Apr 14. [PubMed:15911239 ]
    8. Cynober LA: Plasma amino acid levels with a note on membrane transport: characteristics, regulation, and metabolic significance. Nutrition. 2002 Sep;18(9):761-6. [PubMed:12297216 ]
    9. Rainesalo S, Keranen T, Palmio J, Peltola J, Oja SS, Saransaari P: Plasma and cerebrospinal fluid amino acids in epileptic patients. Neurochem Res. 2004 Jan;29(1):319-24. [PubMed:14992292 ]
    10. Deng C, Shang C, Hu Y, Zhang X: Rapid diagnosis of phenylketonuria and other aminoacidemias by quantitative analysis of amino acids in neonatal blood spots by gas chromatography-mass spectrometry. J Chromatogr B Analyt Technol Biomed Life Sci. 2002 Jul 25;775(1):115-20. [PubMed:12101068 ]
    11. Wannemacher RW Jr, Klainer AS, Dinterman RE, Beisel WR: The significance and mechanism of an increased serum phenylalanine-tyrosine ratio during infection. Am J Clin Nutr. 1976 Sep;29(9):997-1006. [PubMed:822705 ]
    12. Doellgast GJ, Meis PJ: Use of specific inhibitors to disciminate alkaline phosphatase isoenzymes originating from human liver, placenta and intestine: absence of meconial alkaline phosphatase in maternal serum. Clin Chem. 1979 Jul;25(7):1230-3. [PubMed:455643 ]
    13. Kersemans V, Cornelissen B, Kersemans K, Bauwens M, Achten E, Dierckx RA, Mertens J, Slegers G: In vivo characterization of 123/125I-2-iodo-L-phenylalanine in an R1M rhabdomyosarcoma athymic mouse model as a potential tumor tracer for SPECT. J Nucl Med. 2005 Mar;46(3):532-9. [PubMed:15750170 ]
    14. Klassen P, Furst P, Schulz C, Mazariegos M, Solomons NW: Plasma free amino acid concentrations in healthy Guatemalan adults and in patients with classic dengue. Am J Clin Nutr. 2001 Mar;73(3):647-52. [PubMed:11237944 ]
    15. van Spronsen FJ, Hoeksma M, Reijngoud DJ: Brain dysfunction in phenylketonuria: is phenylalanine toxicity the only possible cause? J Inherit Metab Dis. 2009 Feb;32(1):46-51. doi: 10.1007/s10545-008-0946-2. Epub 2009 Jan 13. [PubMed:19191004 ]
    16. Adler-Abramovich L, Vaks L, Carny O, Trudler D, Magno A, Caflisch A, Frenkel D, Gazit E: Phenylalanine assembly into toxic fibrils suggests amyloid etiology in phenylketonuria. Nat Chem Biol. 2012 Aug;8(8):701-6. doi: 10.1038/nchembio.1002. Epub 2012 Jun 17. [PubMed:22706200 ]

    Only showing the first 10 proteins. There are 19 proteins in total.

    Enzymes

    General function:
    Involved in monooxygenase activity
    Specific function:
    Plays an important role in the physiology of adrenergic neurons.
    Gene Name:
    TH
    Uniprot ID:
    P07101
    Molecular weight:
    55611.26
    References
    1. Fukami MH, Haavik J, Flatmark T: Phenylalanine as substrate for tyrosine hydroxylase in bovine adrenal chromaffin cells. Biochem J. 1990 Jun 1;268(2):525-8. [PubMed:1973034 ]
    2. Ogawa S, Ichinose H: Effect of metals and phenylalanine on the activity of human tryptophan hydroxylase-2: comparison with that on tyrosine hydroxylase activity. Neurosci Lett. 2006 Jul 3;401(3):261-5. Epub 2006 Apr 11. [PubMed:16581181 ]
    3. Schallreuter KU, Kothari S, Hasse S, Kauser S, Lindsey NJ, Gibbons NC, Hibberts N, Wood JM: In situ and in vitro evidence for DCoH/HNF-1 alpha transcription of tyrosinase in human skin melanocytes. Biochem Biophys Res Commun. 2003 Feb 7;301(2):610-6. [PubMed:12565907 ]
    4. McQuade PS, Juorio AV: The effect of various amino acids and drugs on the para- and meta-hydroxyphenylacetic acid concentrations in the mouse caudate nucleus. Neurochem Res. 1983 Jul;8(7):903-12. [PubMed:6621777 ]
    5. Fusetti F, Erlandsen H, Flatmark T, Stevens RC: Structure of tetrameric human phenylalanine hydroxylase and its implications for phenylketonuria. J Biol Chem. 1998 Jul 3;273(27):16962-7. [PubMed:9642259 ]
    6. Chen X, Ji ZL, Chen YZ: TTD: Therapeutic Target Database. Nucleic Acids Res. 2002 Jan 1;30(1):412-5. [PubMed:11752352 ]
    General function:
    Involved in nucleotide binding
    Specific function:
    Not Available
    Gene Name:
    FARSA
    Uniprot ID:
    Q9Y285
    Molecular weight:
    57563.225
    Reactions
    Adenosine triphosphate + L-Phenylalanine + tRNA(Phe) → Adenosine monophosphate + Pyrophosphate + L-phenylalanyl-tRNA(Phe)details
    Adenosine triphosphate + L-Phenylalanine + tRNA(Phe) → Adenosine monophosphate + Pyrophosphate + L-Phenylalanyl-tRNA(Phe)details
    References
    1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
    2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [PubMed:17016423 ]
    3. Kodama K, Fukuzawa S, Sakamoto K, Nakayama H, Kigawa T, Yabuki T, Matsuda N, Shirouzu M, Takio K, Tachibana K, Yokoyama S: A new protein engineering approach combining chemistry and biology, part I; site-specific incorporation of 4-iodo-L-phenylalanine in vitro by using misacylated suppressor tRNAPhe. Chembiochem. 2006 Oct;7(10):1577-81. [PubMed:16969782 ]
    4. Kotik-Kogan O, Moor N, Tworowski D, Safro M: Structural basis for discrimination of L-phenylalanine from L-tyrosine by phenylalanyl-tRNA synthetase. Structure. 2005 Dec;13(12):1799-807. [PubMed:16338408 ]
    General function:
    Involved in nucleotide binding
    Specific function:
    Catalyzes direct attachment of p-Tyr (Tyr) to tRNAPhe. Permits also, with a lower efficiency, the attachment of m-Tyr to tRNAPhe, thereby opening the way for delivery of the misacylated tRNA to the ribosome and incorporation of ROS-damaged amino acid into proteins.
    Gene Name:
    FARS2
    Uniprot ID:
    O95363
    Molecular weight:
    52356.21
    Reactions
    Adenosine triphosphate + L-Phenylalanine + tRNA(Phe) → Adenosine monophosphate + Pyrophosphate + L-phenylalanyl-tRNA(Phe)details
    Adenosine triphosphate + L-Phenylalanine + tRNA(Phe) → Adenosine monophosphate + Pyrophosphate + L-Phenylalanyl-tRNA(Phe)details
    References
    1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
    2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [PubMed:17016423 ]
    3. Kodama K, Fukuzawa S, Sakamoto K, Nakayama H, Kigawa T, Yabuki T, Matsuda N, Shirouzu M, Takio K, Tachibana K, Yokoyama S: A new protein engineering approach combining chemistry and biology, part I; site-specific incorporation of 4-iodo-L-phenylalanine in vitro by using misacylated suppressor tRNAPhe. Chembiochem. 2006 Oct;7(10):1577-81. [PubMed:16969782 ]
    4. Kotik-Kogan O, Moor N, Tworowski D, Safro M: Structural basis for discrimination of L-phenylalanine from L-tyrosine by phenylalanyl-tRNA synthetase. Structure. 2005 Dec;13(12):1799-807. [PubMed:16338408 ]
    General function:
    Involved in 1-aminocyclopropane-1-carboxylate synthase activity
    Specific function:
    Transaminase involved in tyrosine breakdown. Converts tyrosine to p-hydroxyphenylpyruvate. Can catalyze the reverse reaction, using glutamic acid, with 2-oxoglutarate as cosubstrate (in vitro). Has much lower affinity and transaminase activity towards phenylalanine.
    Gene Name:
    TAT
    Uniprot ID:
    P17735
    Molecular weight:
    50398.895
    Reactions
    L-Phenylalanine + Oxoglutaric acid → Phenylpyruvic acid + L-Glutamic aciddetails
    References
    1. Luong TN, Kirsch JF: A continuous coupled spectrophotometric assay for tyrosine aminotransferase activity with aromatic and other nonpolar amino acids. Anal Biochem. 1997 Nov 1;253(1):46-9. [PubMed:9356140 ]
    2. Rege AA: Purification and characterization of a tyrosine aminotransferase from Crithidia fasciculata. Mol Biochem Parasitol. 1987 Aug;25(1):1-9. [PubMed:2890101 ]
    3. De-Eknamkul W, Ellis BE: Behavior of Free Aromatic Amino Acid Pools in Rosmarinic Acid-Producing Cell Cultures of Anchusa officinalis L. Plant Physiol. 1989 Feb;89(2):429-33. [PubMed:16666560 ]
    4. Patrizio M, Colucci M, Levi G: Human immunodeficiency virus type 1 Tat protein decreases cyclic AMP synthesis in rat microglia cultures. J Neurochem. 2001 Apr;77(2):399-407. [PubMed:11299302 ]
    General function:
    Involved in carboxy-lyase activity
    Specific function:
    Catalyzes the decarboxylation of L-3,4-dihydroxyphenylalanine (DOPA) to dopamine, L-5-hydroxytryptophan to serotonin and L-tryptophan to tryptamine.
    Gene Name:
    DDC
    Uniprot ID:
    P20711
    Molecular weight:
    53893.755
    Reactions
    L-Phenylalanine → Phenylethylamine + Carbon dioxidedetails
    General function:
    Involved in peroxidase activity
    Specific function:
    May contribute to airway host defense against infection.
    Gene Name:
    LPO
    Uniprot ID:
    P22079
    Molecular weight:
    70983.6
    General function:
    Involved in amino acid binding
    Specific function:
    Not Available
    Gene Name:
    PAH
    Uniprot ID:
    P00439
    Molecular weight:
    51861.565
    Reactions
    L-Phenylalanine + L-erythro-tetrahydrobiopterin + Oxygen → L-Tyrosine + 4a-Hydroxytetrahydrobiopterindetails
    L-erythro-tetrahydrobiopterin + L-Phenylalanine + Oxygen → 4a-Carbinolamine tetrahydrobiopterin + L-Tyrosine + Waterdetails
    References
    1. Thorolfsson M, Ibarra-Molero B, Fojan P, Petersen SB, Sanchez-Ruiz JM, Martinez A: L-phenylalanine binding and domain organization in human phenylalanine hydroxylase: a differential scanning calorimetry study. Biochemistry. 2002 Jun 18;41(24):7573-85. [PubMed:12056888 ]
    2. Pueschel SM, Boylan J, Ellenbogen R: Studies on experimentally induced hyperphenylalaninemia. J Ment Defic Res. 1988 Aug;32 ( Pt 4):309-19. [PubMed:2975336 ]
    3. Nagasaki Y, Matsubara Y, Takano H, Fujii K, Senoo M, Akanuma J, Takahashi K, Kure S, Hara M, Kanegae Y, Saito I, Narisawa K: Reversal of hypopigmentation in phenylketonuria mice by adenovirus-mediated gene transfer. Pediatr Res. 1999 Apr;45(4 Pt 1):465-73. [PubMed:10203136 ]
    4. Fusetti F, Erlandsen H, Flatmark T, Stevens RC: Structure of tetrameric human phenylalanine hydroxylase and its implications for phenylketonuria. J Biol Chem. 1998 Jul 3;273(27):16962-7. [PubMed:9642259 ]
    5. Stokka AJ, Flatmark T: Substrate-induced conformational transition in human phenylalanine hydroxylase as studied by surface plasmon resonance analyses: the effect of terminal deletions, substrate analogues and phosphorylation. Biochem J. 2003 Feb 1;369(Pt 3):509-18. [PubMed:12379147 ]
    General function:
    Involved in antioxidant activity
    Specific function:
    Involved in redox regulation of the cell. Can reduce H(2)O(2) and short chain organic, fatty acid, and phospholipid hydroperoxides. May play a role in the regulation of phospholipid turnover as well as in protection against oxidative injury.
    Gene Name:
    PRDX6
    Uniprot ID:
    P30041
    Molecular weight:
    25034.715
    Reactions
    L-Phenylalanine + Oxygen → 2-Phenylacetamide + Carbon dioxidedetails
    General function:
    Involved in peroxidase activity
    Specific function:
    Part of the host defense system of polymorphonuclear leukocytes. It is responsible for microbicidal activity against a wide range of organisms. In the stimulated PMN, MPO catalyzes the production of hypohalous acids, primarily hypochlorous acid in physiologic situations, and other toxic intermediates that greatly enhance PMN microbicidal activity.
    Gene Name:
    MPO
    Uniprot ID:
    P05164
    Molecular weight:
    83867.71
    General function:
    Involved in transferase activity, transferring nitrogenous groups
    Specific function:
    Plays a key role in amino acid metabolism (By similarity).
    Gene Name:
    GOT1
    Uniprot ID:
    P17174
    Molecular weight:
    46247.14
    Reactions
    L-Phenylalanine + Oxoglutaric acid → Phenylpyruvic acid + L-Glutamic aciddetails

    Transporters

    General function:
    Involved in transport
    Specific function:
    Sodium-independent, high-affinity transport of small and large neutral amino acids such as alanine, serine, threonine, cysteine, phenylalanine, tyrosine, leucine, arginine and tryptophan, when associated with SLC3A2/4F2hc. Acts as an amino acid exchanger. Has higher affinity for L-phenylalanine than LAT1 but lower affinity for glutamine and serine. L-alanine is transported at physiological concentrations. Plays a role in basolateral (re)absorption of neutral amino acids. Involved in the uptake of methylmercury (MeHg) when administered as the L-cysteine or D,L-homocysteine complexes, and hence plays a role in metal ion homeostasis and toxicity. Involved in the cellular activity of small molecular weight nitrosothiols, via the stereoselective transport of L-nitrosocysteine (L-CNSO) across the transmembrane. Plays an essential role in the reabsorption of neutral amino acids from the epithelial cells to the bloodstream in the kidney
    Gene Name:
    SLC7A8
    Uniprot ID:
    Q9UHI5
    Molecular weight:
    58381.1
    References
    1. Shennan DB, Calvert DT, Travers MT, Kudo Y, Boyd CA: A study of L-leucine, L-phenylalanine and L-alanine transport in the perfused rat mammary gland: possible involvement of LAT1 and LAT2. Biochim Biophys Acta. 2002 Aug 19;1564(1):133-9. [PubMed:12101005 ]
    2. Rossier G, Meier C, Bauch C, Summa V, Sordat B, Verrey F, Kuhn LC: LAT2, a new basolateral 4F2hc/CD98-associated amino acid transporter of kidney and intestine. J Biol Chem. 1999 Dec 3;274(49):34948-54. [PubMed:10574970 ]
    3. Babu E, Kanai Y, Chairoungdua A, Kim DK, Iribe Y, Tangtrongsup S, Jutabha P, Li Y, Ahmed N, Sakamoto S, Anzai N, Nagamori S, Endou H: Identification of a novel system L amino acid transporter structurally distinct from heterodimeric amino acid transporters. J Biol Chem. 2003 Oct 31;278(44):43838-45. Epub 2003 Aug 20. [PubMed:12930836 ]
    4. Satoh S, Kimura T, Toda M, Maekawa M, Ono S, Narita H, Miyazaki H, Murayama T, Nomura Y: Involvement of L-type-like amino acid transporters in S-nitrosocysteine-stimulated noradrenaline release in the rat hippocampus. J Neurochem. 1997 Nov;69(5):2197-205. [PubMed:9349567 ]
    5. Nemoto T, Shimma N, Horie S, Saito T, Okuma Y, Nomura Y, Murayama T: Involvement of the system L amino acid transporter on uptake of S-nitroso-L-cysteine, an endogenous S-nitrosothiol, in PC12 cells. Eur J Pharmacol. 2003 Jan 1;458(1-2):17-24. [PubMed:12498902 ]
    General function:
    Involved in transmembrane transport
    Specific function:
    Sodium-independent transporter that mediates the update of aromatic acid. Can function as a net efflux pathway for aromatic amino acids in the basosolateral epithelial cells
    Gene Name:
    SLC16A10
    Uniprot ID:
    Q8TF71
    Molecular weight:
    55492.1
    References
    1. Kim DK, Kanai Y, Matsuo H, Kim JY, Chairoungdua A, Kobayashi Y, Enomoto A, Cha SH, Goya T, Endou H: The human T-type amino acid transporter-1: characterization, gene organization, and chromosomal location. Genomics. 2002 Jan;79(1):95-103. [PubMed:11827462 ]
    2. Kim DK, Kanai Y, Chairoungdua A, Matsuo H, Cha SH, Endou H: Expression cloning of a Na+-independent aromatic amino acid transporter with structural similarity to H+/monocarboxylate transporters. J Biol Chem. 2001 May 18;276(20):17221-8. Epub 2001 Feb 20. [PubMed:11278508 ]

    Only showing the first 10 proteins. There are 19 proteins in total.