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Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2016-02-11 01:04:12 UTC
HMDB IDHMDB01301
Secondary Accession Numbers
  • HMDB02240
Metabolite Identification
Common Name1-Pyrroline-5-carboxylic acid
Description1-Pyrroline-5-carboxylic acid is an enamine or an imino acid that forms on spontaneous dehydration of L-glutamate γ-semialdehyde in aqueous solutions. The stereoisomer (S)-1-Pyrroline-5-carboxylate is an intermediate in glutamate metabolism, in arginine degradation and in proline biosynthesis and degradation and it can be converted to or be formed from the three amino acids L-glutamate, L-ornithine and L-proline. In particular, it is synthesized with the oxidation of proline by pyrroline-5-carboxylate reductase 1 (EC 1.5.1.2, PYCR1) or by proline dehydrogenase (EC 1.5.99.8, PRODH) and it is hydrolyzed to L-glutamate by delta-1-pyrroline-5-carboxylate dehydrogenase (EC 1.5.1.12, ALDH4A1). It is also one of the few metabolites that can be a precursor to other metabolites of both the urea cycle and the tricarboxylic acid (TCA) cycle.
Structure
Thumb
Synonyms
ValueSource
3,4-dihydro-2H-Pyrrole-2-carboxylateChEBI
Delta(1)-Pyrroline-5-carboxylic acidChEBI
3,4-dihydro-2H-Pyrrole-2-carboxylic acidGenerator
1-Pyrroline-5-carboxylateGenerator
delta(1)-Pyrroline-5-carboxylateGenerator
δ(1)-pyrroline-5-carboxylateGenerator
δ(1)-pyrroline-5-carboxylic acidGenerator
(S)-1-Pyrroline-5-carboxylateHMDB
D1-Pyrroline-5-carboxylateHMDB
D1-Pyrroline-5-carboxylic acidHMDB
delta(1)Pyrroline-5-carboxylateHMDB
delta-1-Pyrroline-5-carboxylateHMDB
delta-1-Pyrroline-5-carboxylic acidHMDB
DL-1-Pyrroline-5-carboxylateHMDB
DL-1-Pyrroline-5-carboxylic acidHMDB
L-1-Pyrroline-5-carboxylateHMDB
L-delta 1-Pyrroline-5-carboxylateHMDB
Pyrroline 5-carboxylateHMDB
Pyrroline-5-carboxylateHMDB
Chemical FormulaC5H7NO2
Average Molecular Weight113.1146
Monoisotopic Molecular Weight113.047678473
IUPAC Name3,4-dihydro-2H-pyrrole-2-carboxylic acid
Traditional Name1-pyrroline-5-carboxylic acid
CAS Registry Number2906-39-0
SMILES
OC(=O)C1CCC=N1
InChI Identifier
InChI=1S/C5H7NO2/c7-5(8)4-2-1-3-6-4/h3-4H,1-2H2,(H,7,8)
InChI KeyInChIKey=DWAKNKKXGALPNW-UHFFFAOYSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as alpha amino acids and derivatives. These are amino acids in which the amino group is attached to the carbon atom immediately adjacent to the carboxylate group (alpha carbon), or a derivative thereof.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassAmino acids, peptides, and analogues
Direct ParentAlpha amino acids and derivatives
Alternative Parents
Substituents
  • Alpha-amino acid or derivatives
  • Pyrroline carboxylic acid or derivatives
  • Pyrroline carboxylic acid
  • Pyrroline
  • Azacycle
  • Organoheterocyclic compound
  • Organic 1,3-dipolar compound
  • Propargyl-type 1,3-dipolar organic compound
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Hydrocarbon derivative
  • Organooxygen compound
  • Organonitrogen compound
  • Imine
  • Carbonyl group
  • Aliphatic heteromonocyclic compound
Molecular FrameworkAliphatic heteromonocyclic compounds
External DescriptorsNot Available
Ontology
StatusExpected but not Quantified
Origin
  • Endogenous
Biofunction
  • Component of Arginine and proline metabolism
  • Component of Glutamate metabolism
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 Solubility13.1 mg/mLALOGPS
logP-0.01ALOGPS
logP-2.3ChemAxon
logS-0.94ALOGPS
pKa (Strongest Acidic)1.82ChemAxon
pKa (Strongest Basic)6.07ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count1ChemAxon
Polar Surface Area49.66 Å2ChemAxon
Rotatable Bond Count1ChemAxon
Refractivity27.4 m3·mol-1ChemAxon
Polarizability10.7 Å3ChemAxon
Number of Rings1ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
Predicted GC-MSPredicted GC-MS Spectrum - GC-MSNot Available
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-03dj-9700000000-f2bc2275638f96dc74acView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-014i-9100000000-72dff6639c24448bec9cView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-014l-9000000000-28eb1358005b28459debView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-03di-5900000000-0720aa91caee03b9364eView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-02t9-9400000000-cfcc87863f675fb12691View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-00kf-9000000000-a15b91c200295166bf9eView in MoNA
Biological Properties
Cellular Locations
  • Mitochondria
Biofluid LocationsNot Available
Tissue Location
  • Intestine
Pathways
NameSMPDB LinkKEGG Link
2-Hydroxyglutric Aciduria (D And L Form)SMP00136Not Available
4-Hydroxybutyric Aciduria/Succinic Semialdehyde Dehydrogenase DeficiencySMP00243Not Available
Arginine and Proline MetabolismSMP00020map00330
Arginine: Glycine Amidinotransferase Deficiency (AGAT Deficiency)SMP00362Not Available
Creatine deficiency, guanidinoacetate methyltransferase deficiencySMP00504Not Available
Glutamate MetabolismSMP00072map00250
Guanidinoacetate Methyltransferase Deficiency (GAMT Deficiency)SMP00188Not Available
HomocarnosinosisSMP00385Not Available
Hyperinsulinism-Hyperammonemia SyndromeSMP00339Not Available
Hyperornithinemia with gyrate atrophy (HOGA)SMP00505Not Available
Hyperornithinemia-hyperammonemia-homocitrullinuria [HHH-syndrome]SMP00506Not Available
Hyperprolinemia Type ISMP00361Not Available
Hyperprolinemia Type IISMP00360Not Available
L-arginine:glycine amidinotransferase deficiencySMP00507Not Available
Ornithine Aminotransferase Deficiency (OAT Deficiency)SMP00363Not Available
Prolidase Deficiency (PD)SMP00207Not Available
Prolinemia Type IISMP00208Not Available
Succinic semialdehyde dehydrogenase deficiencySMP00567Not Available
Normal Concentrations
Not Available
Abnormal Concentrations
Not Available
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 IDFDB022542
KNApSAcK IDNot Available
Chemspider ID1159
KEGG Compound IDC04322
BioCyc IDl-delta(1)-pyrroline_5-carboxylate
BiGG ID42766
Wikipedia Link1-pyrroline-5-carboxylate
NuGOwiki LinkHMDB01301
Metagene LinkHMDB01301
METLIN ID6145
PubChem Compound1196
PDB IDNot Available
ChEBI ID1372
References
Synthesis ReferenceVogel, Henry J.; Davis, Bernard D. Glutamic g-semialdehyde and D1-pyrroline-5-carboxylic acid, intermediates in the biosynthesis of proline. Journal of the American Chemical Society (1952), 74 109-12.
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. [533224 ]
  2. Humbertclaude V, Rivier F, Roubertie A, Echenne B, Bellet H, Vallat C, Morin D: Is hyperprolinemia type I actually a benign trait? Report of a case with severe neurologic involvement and vigabatrin intolerance. J Child Neurol. 2001 Aug;16(8):622-3. [11510941 ]
  3. Mixson AJ, Phang JM: The uptake of pyrroline 5-carboxylate. Group translocation mediating the transfer of reducing-oxidizing potential. J Biol Chem. 1988 Aug 5;263(22):10720-4. [3392037 ]
  4. Wakabayashi Y: Tissue-selective expression of enzymes of arginine synthesis. Curr Opin Clin Nutr Metab Care. 1998 Jul;1(4):335-9. [10565370 ]
  5. 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. [15214748 ]
  6. Fleming GA, Hagedorn CH, Granger AS, Phang JM: Pyrroline-5-carboxylate in human plasma. Metabolism. 1984 Aug;33(8):739-42. [6748947 ]

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
1-Pyrroline-5-carboxylic acid + NAD(P)(+) + Water → L-Glutamic acid + NAD(P)Hdetails
1-Pyrroline-5-carboxylic acid + NAD + Water → L-Glutamic acid + NADH + Hydrogen Iondetails
1-Pyrroline-5-carboxylic acid + NADP + Water → 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
L-Proline + NAD(P)(+) → 1-Pyrroline-5-carboxylic acid + NAD(P)Hdetails
L-Proline + NAD → 1-Pyrroline-5-carboxylic acid + NADH + Hydrogen Iondetails
L-Proline + NADP → 1-Pyrroline-5-carboxylic 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
L-Proline + NAD(P)(+) → 1-Pyrroline-5-carboxylic acid + NAD(P)Hdetails
L-Proline + NAD → 1-Pyrroline-5-carboxylic acid + NADH + Hydrogen Iondetails
L-Proline + NADP → 1-Pyrroline-5-carboxylic acid + NADPH + Hydrogen Iondetails
General function:
Involved in proline dehydrogenase activity
Specific function:
Converts proline to delta-1-pyrroline-5-carboxylate.
Gene Name:
PRODH
Uniprot ID:
O43272
Molecular weight:
56196.675
Reactions
L-Proline + acceptor → 1-Pyrroline-5-carboxylic acid + reduced acceptordetails
L-Proline + Acceptor → 1-Pyrroline-5-carboxylic acid + Reduced acceptordetails
General function:
Involved in oxidoreductase activity
Specific function:
Not Available
Gene Name:
PYCRL
Uniprot ID:
Q53H96
Molecular weight:
29892.415
Reactions
L-Proline + NAD(P)(+) → 1-Pyrroline-5-carboxylic acid + NAD(P)Hdetails
L-Proline + NAD → 1-Pyrroline-5-carboxylic acid + NADH + Hydrogen Iondetails
L-Proline + NADP → 1-Pyrroline-5-carboxylic acid + NADPH + Hydrogen Iondetails
General function:
Involved in proline dehydrogenase activity
Specific function:
Converts proline to delta-1-pyrroline-5-carboxylate (Probable).
Gene Name:
PRODH2
Uniprot ID:
Q9UF12
Molecular weight:
58870.115
Reactions
L-Proline + acceptor → 1-Pyrroline-5-carboxylic acid + reduced acceptordetails