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Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2014-10-28 22:57:40 UTC
HMDB IDHMDB01369
Secondary Accession NumbersNone
Metabolite Identification
Common NamePyrroline hydroxycarboxylic acid
DescriptionPyrroline hydroxycarboxylic acid is a metabolite identified in the urine of patients with type II hyperprolinemia. (OMIM 239510 ). The urinary excretion of Pyrroline hydroxycarboxylic acid increased in hyperprolinemic patients but not in healthy controls during oral loading of hydroxyproline and hydroxyproline-ornithine. (PMID: 533224 ). Hyperprolinemia type II (HP II) is a rare inherited metabolic disease due to the deficiency of pyroline-5-carboxylate dehydrogenase. It is generally believed to be a benign condition although some patients have neurological problems such as refractory convulsions. (PMID: 15214748 ). The oxidation of pyrroline-carboxylate generates glutamate and pyrroline-hydroxycarboxylate, a reaction catalyzed by hydroxyproline oxidase (PMID: 500817 ).
Structure
Thumb
Synonyms
  1. 3-Hydroxy-L-1-pyrroline-5-carboxylate
  2. L-1-Pyrroline 3-hydroxy-5-carboxylate
  3. L-1-Pyrroline-3-hydroxy-5-carboxylate
  4. L-Delta1-pyrroline 3-hydroxy-5-carboxylate
  5. Pyrroline-hydroxy-carboxylate
Chemical FormulaC5H7NO3
Average Molecular Weight129.114
Monoisotopic Molecular Weight129.042593095
IUPAC Name4-hydroxy-3,4-dihydro-2H-pyrrole-2-carboxylic acid
Traditional Name4-hydroxy-3,4-dihydro-2H-pyrrole-2-carboxylic acid
CAS Registry Number22573-88-2
SMILES
OC1CC(N=C1)C(O)=O
InChI Identifier
InChI=1S/C5H7NO3/c7-3-1-4(5(8)9)6-2-3/h2-4,7H,1H2,(H,8,9)
InChI KeyWFOFKRKDDKGRIK-UHFFFAOYSA-N
Chemical Taxonomy
KingdomOrganic Compounds
Super ClassAliphatic Heteromonocyclic Compounds
ClassPyrrolines
Sub ClassN/A
Other Descriptors
  • 1-pyrrolinecarboxylic acid(ChEBI)
  • Aliphatic Heteromonocyclic Compounds
Substituents
  • Carboxylic Acid
  • Imine
  • Secondary Alcohol
Direct ParentPyrrolines
Ontology
StatusDetected and Not Quantified
Origin
  • Endogenous
Biofunction
  • Protein synthesis, amino acid biosynthesis
ApplicationNot Available
Cellular locations
  • Mitochondria
Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility33.9 g/LALOGPS
logP-1.1ALOGPS
logP-1.5ChemAxon
logS-0.58ALOGPS
pKa (Strongest Acidic)3.72ChemAxon
pKa (Strongest Basic)2.59ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count4ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area69.89ChemAxon
Rotatable Bond Count1ChemAxon
Refractivity28.85ChemAxon
Polarizability11.6ChemAxon
Spectra
SpectraMS/MS
Biological Properties
Cellular Locations
  • Mitochondria
Biofluid Locations
  • Saliva
Tissue LocationNot Available
Pathways
NameSMPDB LinkKEGG Link
Arginine and Proline MetabolismSMP00020map00330
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)BothNormal details
Abnormal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)BothOral cancer details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)FemaleBreast cancer details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Not SpecifiedPancreatic cancer details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Not SpecifiedPeriodontal diseases details
Associated Disorders and Diseases
Disease ReferencesNone
Associated OMIM IDsNone
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB022582
KNApSAcK IDNot Available
Chemspider ID1030
KEGG Compound IDC04281
BioCyc IDPYRROLINE-HYDROXY-CARBOXYLATE
BiGG ID43511
Wikipedia LinkNot Available
NuGOwiki LinkHMDB01369
Metagene LinkHMDB01369
METLIN ID6196
PubChem Compound1059
PDB IDNot Available
ChEBI ID27391
References
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Simila S: Hydroxyproline metabolism in type II hyperprolinaemia. Ann Clin Biochem. 1979 Jul;16(4):177-81. Pubmed: 533224
  2. Onenli-Mungan N, Yuksel B, Elkay M, Topaloglu AK, Baykal T, Ozer G: Type II hyperprolinemia: a case report. Turk J Pediatr. 2004 Apr-Jun;46(2):167-9. Pubmed: 15214748
  3. Valle D, Goodman SI, Harris SC, Phang JM: Genetic evidence for a common enzyme catalyzing the second step in the degradation of proline and hydroxyproline. J Clin Invest. 1979 Nov;64(5):1365-70. Pubmed: 500817

Enzymes

General function:
Involved in oxidoreductase activity
Specific function:
Irreversible conversion of delta-1-pyrroline-5-carboxylate (P5C), derived either from proline or ornithine, to glutamate. This is a necessary step in the pathway interconnecting the urea and tricarboxylic acid cycles. The preferred substrate is glutamic gamma-semialdehyde, other substrates include succinic, glutaric and adipic semialdehydes.
Gene Name:
ALDH4A1
Uniprot ID:
P30038
Molecular weight:
55117.24
Reactions
Pyrroline hydroxycarboxylic acid + NAD + Water → 4-Hydroxy-L-glutamic acid + NADH + Hydrogen Iondetails
Pyrroline hydroxycarboxylic acid + NADP + Water → 4-Hydroxy-L-glutamic acid + NADPH + Hydrogen Iondetails
General function:
Involved in oxidoreductase activity
Specific function:
Housekeeping enzyme that catalyzes the last step in proline biosynthesis. Can utilize both NAD and NADP, but has higher affinity for NAD. Involved in the cellular response to oxidative stress.
Gene Name:
PYCR1
Uniprot ID:
P32322
Molecular weight:
33360.27
Reactions
4-Hydroxyproline + NAD → Pyrroline hydroxycarboxylic acid + NADH + Hydrogen Iondetails
4-Hydroxyproline + NADP → Pyrroline hydroxycarboxylic acid + NADPH + Hydrogen Iondetails
General function:
Involved in oxidoreductase activity
Specific function:
Housekeeping enzyme that catalyzes the last step in proline biosynthesis. In some cell types, such as erythrocytes, its primary function may be the generation of NADP(+). Can utilize both NAD and NADP. Has higher affinity for NADP, but higher catalytic efficiency with NADH.
Gene Name:
PYCR2
Uniprot ID:
Q96C36
Molecular weight:
25867.975
Reactions
4-Hydroxyproline + NAD → Pyrroline hydroxycarboxylic acid + NADH + Hydrogen Iondetails
4-Hydroxyproline + NADP → Pyrroline hydroxycarboxylic acid + NADPH + Hydrogen Iondetails
General function:
Involved in oxidoreductase activity
Specific function:
Not Available
Gene Name:
PYCRL
Uniprot ID:
Q53H96
Molecular weight:
29892.415
Reactions
4-Hydroxyproline + NAD → Pyrroline hydroxycarboxylic acid + NADH + Hydrogen Iondetails
4-Hydroxyproline + NADP → Pyrroline hydroxycarboxylic acid + NADPH + Hydrogen Iondetails