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Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2016-09-22 21:06:22 UTC
HMDB IDHMDB00557
Secondary Accession Numbers
  • HMDB02291
Metabolite Identification
Common NameL-Alloisoleucine
DescriptionL-alloisoleucine is a branched chain amino acid and is a stereo-isomer of L-isoleucine. It is a common constituent of human plasma (albeit at low levels). L-alloisoleucine is produced as a byproduct of isoleucine transamination. L-Isoleucine is one of the four common amino acids (the 3 others being threonine, hydroxyproline, and hydroxylysine) that have two asymmetric carbon atoms which produce four structural possibilities for the same chemical composition. L-alloisoleucine differs from L-isoleucine by having having different stereochemistry around its beta carbon. Plasma L-alloisoleucine, which is derived from L-isoleucine in vivo, can be used for the diagnosis of maple syrup urine disease (MSUD). Indeed, plasma L-alloisoleucine levels above 5 umol/L is the most specific and most sensitive diagnostic marker for all forms of MSUD (PMID: 10508118 ). In MSUD the degradation of the essential branched-chain L-amino acids leucine, valine, and isoleucine and their derived 2-oxoacids is impaired because of an inherited deficiency in branched-chain 2-oxoacid dehydrogenase complex (EC 1.2.4.4) activity. The accumulation of branched-chain compounds in blood and other body fluids can exert neurotoxic effects.
Structure
Thumb
Synonyms
ValueSource
(2S,3R)-2-amino-3-Methylpentanoic acidChEBI
AlleChEBI
allo-L-IsoleucineChEBI
L(+)-AlloisoleucineChEBI
threo-3-Methyl-L-norvalineChEBI
threo-L-IsoleucineChEBI
(2S,3R)-2-amino-3-MethylpentanoateGenerator
(3R)-LS-IsoleucineHMDB
2-amino-3-Methyl-[S-(r*,s*)]-pentanoateHMDB
2-amino-3-Methyl-[S-(r*,s*)]-pentanoic acidHMDB
allo-IsoleucineHMDB
L-allo-IsoleucineHMDB
[S-(R*,s*)]-2-amino-3-methylpentanoateHMDB
[S-(R*,s*)]-2-amino-3-methylpentanoic acidHMDB
AlloisoleucineMeSH
Isoleucine, L isomerMeSH
Isoleucine, L-isomerMeSH
L-Isomer isoleucineMeSH
L-IsoleucineMeSH
IsoleucineMeSH
Chemical FormulaC6H13NO2
Average Molecular Weight131.1729
Monoisotopic Molecular Weight131.094628665
IUPAC Name(2S,3R)-2-amino-3-methylpentanoic acid
Traditional Nameallo-isoleucine
CAS Registry Number1509-34-8
SMILES
CC[C@@H](C)[C@H](N)C(O)=O
InChI Identifier
InChI=1S/C6H13NO2/c1-3-4(2)5(7)6(8)9/h4-5H,3,7H2,1-2H3,(H,8,9)/t4-,5+/m1/s1
InChI KeyInChIKey=AGPKZVBTJJNPAG-UHNVWZDZSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of chemical entities known as isoleucine and derivatives. These are compounds containing isoleucine or a derivative thereof resulting from reaction of isoleucine at the amino group or the carboxy group, or from the replacement of any hydrogen of glycine by a heteroatom.
KingdomChemical entities
Super ClassOrganic compounds
ClassOrganic acids and derivatives
Sub ClassCarboxylic acids and derivatives
Direct ParentIsoleucine and derivatives
Alternative Parents
Substituents
  • Isoleucine or derivatives
  • Alpha-amino acid
  • L-alpha-amino acid
  • Branched fatty acid
  • Methyl-branched fatty acid
  • Fatty acid
  • Fatty acyl
  • Amino acid
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Organic oxide
  • Organopnictogen compound
  • Primary amine
  • Organooxygen compound
  • Organonitrogen compound
  • Primary aliphatic amine
  • Carbonyl group
  • Organic oxygen compound
  • Amine
  • Organic nitrogen compound
  • Hydrocarbon derivative
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
StatusDetected and Quantified
Origin
  • Endogenous
BiofunctionNot Available
ApplicationNot Available
Cellular locations
  • Cytoplasm
Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point60 - 64 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility34.4 mg/mL at 25 °CNot Available
LogP-1.70HANSCH,C ET AL. (1995)
Predicted Properties
PropertyValueSource
Water Solubility114.0 mg/mLALOGPS
logP-1.7ALOGPS
logP-1.5ChemAxon
logS-0.06ALOGPS
pKa (Strongest Acidic)2.79ChemAxon
pKa (Strongest Basic)9.59ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area63.32 Å2ChemAxon
Rotatable Bond Count3ChemAxon
Refractivity34.09 m3·mol-1ChemAxon
Polarizability14.23 Å3ChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-001i-3900000000-703d0f350ebca58a7eb1View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-00ko-9000000000-b7c643e18a4283fc88e4View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-052f-9000000000-6b799d2abf4132bac974View in MoNA
1D NMR1H NMR SpectrumNot Available
2D NMR[1H,13C] 2D NMR SpectrumNot Available
Biological Properties
Cellular Locations
  • Cytoplasm
Biofluid Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Urine
Tissue LocationNot Available
PathwaysNot Available
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0-4 uMNewborn (0-30 days old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified0-4 uMInfant (1 - 3 months old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified0-4 uMChildren (3 months - 6 years old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified0-4 uMChildren (6 - 18 years old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified1.9 +/- 0.6 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified1.6 +/- 0.4 uMChildren (1-13 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified0-0.5 uMChildren (0 - 10 years old)Both
Normal
    • BC Children's Hos...
details
Cerebrospinal Fluid (CSF)Detected and Quantified0-0.5 uMAdolescent (>11 years old)Both
Normal
    • BC Children's Hos...
details
FecesDetected but not QuantifiedNot ApplicableNot SpecifiedBoth
Normal
details
FecesDetected but not QuantifiedNot ApplicableAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified0 - 0.11 umol/mmol creatinineInfant (1 - 6 months old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified0 - 0.11 umol/mmol creatinineInfant (6 months - <1 year old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified0 - 0.23 umol/mmol creatinineChildren (1 - 2 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified0 - 0.23 umol/mmol creatinineChildren (2 - 4 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified0 - 0.23 umol/mmol creatinineChildren (4 - 13 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified0 - 0.23 umol/mmol creatinineAdolescent (13 - 21 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified0 - 0.68 umol/mmol creatinineAdult (>21 years old)Both
Normal
    • BC Children's Hos...
details
Abnormal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified127 +/- 1 uMChildren (1-13 years old)BothMaple syrup urine disease (MSUD) details
UrineDetected and Quantified1.524 +/- 0.425 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Mordechai, Hien, ...
details
Associated Disorders and Diseases
Disease References
Maple syrup urine disease
  1. Schadewaldt P, Bodner-Leidecker A, Hammen HW, Wendel U: Significance of L-alloisoleucine in plasma for diagnosis of maple syrup urine disease. Clin Chem. 1999 Oct;45(10):1734-40. [10508118 ]
Associated OMIM IDs
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB022117
KNApSAcK IDNot Available
Chemspider ID89698
KEGG Compound IDNot Available
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
NuGOwiki LinkHMDB00557
Metagene LinkHMDB00557
METLIN IDNot Available
PubChem Compound99288
PDB IDIIL
ChEBI ID43433
References
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Korman SH, Andresen BS, Zeharia A, Gutman A, Boneh A, Pitt JJ: 2-ethylhydracrylic aciduria in short/branched-chain acyl-CoA dehydrogenase deficiency: application to diagnosis and implications for the R-pathway of isoleucine oxidation. Clin Chem. 2005 Mar;51(3):610-7. Epub 2004 Dec 22. [15615815 ]
  2. Schadewaldt P, Bodner-Leidecker A, Hammen HW, Wendel U: Significance of L-alloisoleucine in plasma for diagnosis of maple syrup urine disease. Clin Chem. 1999 Oct;45(10):1734-40. [10508118 ]