Human Metabolome Database Version 3.5

Showing metabocard for Pyrroline hydroxycarboxylic acid (HMDB01369)

Record Information
Version 3.5
Creation Date 2005-11-16 08:48:42 -0700
Update Date 2013-05-29 13:31:19 -0600
HMDB ID HMDB01369
Secondary Accession Numbers None
Metabolite Identification
Common Name Pyrroline hydroxycarboxylic acid
Description Pyrroline hydroxycarboxylic acid is a metabolite identified in the urine of patients with type II hyperprolinemia. (OMIM 239510 Link_out). 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 Link_out). 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 Link_out). The oxidation of pyrroline-carboxylate generates glutamate and pyrroline-hydroxycarboxylate, a reaction catalyzed by hydroxyproline oxidase (PMID: 500817 Link_out).
Structure Thumb
Download: MOL | SDF | PDB | SMILES | InChI
Display: 2D Structure | 3D Structure
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 Formula C5H7NO3
Average Molecular Weight 129.114
Monoisotopic Molecular Weight 129.042593095
IUPAC Name 4-hydroxy-3,4-dihydro-2H-pyrrole-2-carboxylic acid
Traditional IUPAC Name 4-hydroxy-3,4-dihydro-2H-pyrrole-2-carboxylic acid
CAS Registry Number 22573-88-2
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 Key WFOFKRKDDKGRIK-UHFFFAOYSA-N
Chemical Taxonomy
Kingdom Organic Compounds
Super Class Aliphatic Heteromonocyclic Compounds
Class Pyrrolines
Sub Class N/A
Other Descriptors
  • 1-pyrrolinecarboxylic acid(ChEBI)
  • Aliphatic Heteromonocyclic Compounds
Substituents
  • Carboxylic Acid
  • Imine
  • Secondary Alcohol
Direct Parent Pyrrolines
Ontology
Status Expected and Not Quantified
Origin
  • Endogenous
Biofunction
  • Protein synthesis, amino acid biosynthesis
Application Not Available
Cellular locations
  • Mitochondria
Physical Properties
State Solid
Experimental Properties
Property Value Reference
Melting Point Not Available Not Available
Boiling Point Not Available Not Available
Water Solubility Not Available Not Available
LogP Not Available Not Available
Predicted Properties
Property Value Source
Water Solubility 33.9 g/L ALOGPS
LogP -1.10 ALOGPS
LogP -1.5 ChemAxon
LogS -0.58 ALOGPS
pKa (strongest acidic) 3.72 ChemAxon
pKa (strongest basic) 2.59 ChemAxon
Hydrogen Acceptor Count 4 ChemAxon
Hydrogen Donor Count 2 ChemAxon
Polar Surface Area 69.89 A2 ChemAxon
Rotatable Bond Count 1 ChemAxon
Refractivity 28.85 ChemAxon
Polarizability 11.6 ChemAxon
Formal Charge 0 ChemAxon
Physiological Charge -1 ChemAxon
Spectra
MS/MS Spectrum Quattro_QQQ 10
Biological Properties
Cellular Locations
  • Mitochondria
Biofluid Locations Not Available
Tissue Location Not Available
Pathways
Name SMPDB Link KEGG Link
Arginine and Proline Metabolism SMP00020 map00330 Link_out
Normal Concentrations
Not Available
Abnormal Concentrations
Not Available
Associated Disorders and Diseases
Disease References None
Associated OMIM IDs None
DrugBank ID Not Available
DrugBank Metabolite ID Not Available
Phenol Explorer Compound ID Not Available
Phenol Explorer Metabolite ID Not Available
FoodDB ID FDB022582
KNApSAcK ID Not Available
Chemspider ID 1030 Link_out
KEGG Compound ID C04281 Link_out
BioCyc ID PYRROLINE-HYDROXY-CARBOXYLATE Link_out
BiGG ID 43511 Link_out
Wikipedia Link Not Available
NuGOwiki Link HMDB01369 Link_out
Metagene Link HMDB01369 Link_out
METLIN ID 6196 Link_out
PubChem Compound 1059 Link_out
PDB ID Not Available
ChEBI ID 27391 Link_out
References
Synthesis Reference Not 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 Link_out
  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 Link_out
  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 Link_out

Enzymes
Name: Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial
Reactions:
Pyrroline hydroxycarboxylic acid + NAD + Water unknown 4-Hydroxy-L-glutamic acid + NADH + Hydrogen Ion details
Pyrroline hydroxycarboxylic acid + NADP + Water unknown 4-Hydroxy-L-glutamic acid + NADPH + Hydrogen Ion details
Gene Name: ALDH4A1
Uniprot ID: P30038 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Pyrroline-5-carboxylate reductase 1, mitochondrial
Reactions:
4-Hydroxyproline + NAD unknown Pyrroline hydroxycarboxylic acid + NADH + Hydrogen Ion details
4-Hydroxyproline + NADP unknown Pyrroline hydroxycarboxylic acid + NADPH + Hydrogen Ion details
Gene Name: PYCR1
Uniprot ID: P32322 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Pyrroline-5-carboxylate reductase 2
Reactions:
4-Hydroxyproline + NAD unknown Pyrroline hydroxycarboxylic acid + NADH + Hydrogen Ion details
4-Hydroxyproline + NADP unknown Pyrroline hydroxycarboxylic acid + NADPH + Hydrogen Ion details
Gene Name: PYCR2
Uniprot ID: Q96C36 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Pyrroline-5-carboxylate reductase 3
Reactions:
4-Hydroxyproline + NAD unknown Pyrroline hydroxycarboxylic acid + NADH + Hydrogen Ion details
4-Hydroxyproline + NADP unknown Pyrroline hydroxycarboxylic acid + NADPH + Hydrogen Ion details
Gene Name: PYCRL
Uniprot ID: Q53H96 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA