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Showing metabocard for 1-Pyrroline-5-carboxylic acid (HMDB0001301)

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IdentificationTaxonomyOntologyPhysical propertiesSpectraBiological propertiesConcentrationsLinksReferencesenzymes (6) Show 6 proteinsXML
enzymes (6) Show 6 proteins
Record Information
Version5.0
StatusDetected but not Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2021-09-14 15:45:29 UTC
HMDB IDHMDB0001301
Secondary Accession Numbers
  • HMDB0002240
  • HMDB01301
  • HMDB02240
Metabolite Identification
Common Name1-Pyrroline-5-carboxylic acid
Description1-Pyrroline-5-carboxylic acid (CAS: 2906-39-0) is an enamine or an imino acid that forms upon the spontaneous dehydration of L-glutamate gamma-semialdehyde in aqueous solutions. The stereoisomer (S)-1-pyrroline-5-carboxylate is an intermediate in glutamate metabolism, arginine degradation, and proline biosynthesis and degradation. It can also be converted into or be formed from three amino acids: L-glutamate, L-ornithine, and L-proline. In particular, it is synthesized via the oxidation of proline by pyrroline-5-carboxylate reductase 1 (PYCR1) (EC 1.5.1.2) or by proline dehydrogenase (PRODH) (EC 1.5.99.8). It is hydrolyzed into L-glutamate by delta-1-pyrroline-5-carboxylate dehydrogenase (ALDH4A1) (EC 1.5.1.12). It is also one of the few metabolites that can act as a precursor to other metabolites of both the urea cycle and the tricarboxylic acid (TCA) cycle. Under certain conditions, pyrroline-5-carboxylate can act as a neurotoxin and a metabotoxin. A neurotoxin causes damage to nerve cells and nerve tissues. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of pyrroline-5-carboxylate are associated with at least five inborn errors of metabolism, including hyperprolinemia type I, hyperprolinemia type II, iminoglycinuria, prolinemia type II, and pyruvate carboxylase deficiency. Hyperprolinemia type II results in high levels of pyrroline-5-carboxylate. People with hyperprolinemia type II have signs and symptoms that vary in severity, but they are more likely than type I to have seizures or intellectual disability. Pyrroline-5-carboxylate is highly reactive and excess quantities have been shown to cause cell death and apoptosis (PMID: 15548746 ).
Structure
Data?1600107943
MOLSDF3D SDFPDB3D PDBSMILESInChI

MOL for HMDB0001301 (1-Pyrroline-5-carboxylic acid)


  Mrv1652309142020202D          

  8  8  0  0  0  0            999 V2000
    0.6685    0.1811    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.3836   -0.2303    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    0.6685    1.0054    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.7162    0.2642    0.0000 N   0  0  0  0  0  0  0  0  0  0  0  0
   -1.3836   -0.2207    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1287   -1.0053    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.3037   -1.0053    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.0488   -0.2207    0.0000 C   0  0  1  0  0  0  0  0  0  0  0  0
  1  2  1  0  0  0  0
  1  3  2  0  0  0  0
  5  6  1  0  0  0  0
  6  7  1  0  0  0  0
  7  8  1  0  0  0  0
  4  5  2  0  0  0  0
  4  8  1  0  0  0  0
  8  1  1  6  0  0  0
M  END
Download

3D MOL for HMDB0001301 (1-Pyrroline-5-carboxylic acid)

Download
3D SDF for HMDB0001301 (1-Pyrroline-5-carboxylic acid)

  Mrv1652309142020202D          

  8  8  0  0  0  0            999 V2000
    0.6685    0.1811    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.3836   -0.2303    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    0.6685    1.0054    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.7162    0.2642    0.0000 N   0  0  0  0  0  0  0  0  0  0  0  0
   -1.3836   -0.2207    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1287   -1.0053    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.3037   -1.0053    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.0488   -0.2207    0.0000 C   0  0  1  0  0  0  0  0  0  0  0  0
  1  2  1  0  0  0  0
  1  3  2  0  0  0  0
  5  6  1  0  0  0  0
  6  7  1  0  0  0  0
  7  8  1  0  0  0  0
  4  5  2  0  0  0  0
  4  8  1  0  0  0  0
  8  1  1  6  0  0  0
M  END
> <DATABASE_ID>
HMDB0001301

> <DATABASE_NAME>
hmdb

> <SMILES>
OC(=O)[C@@H]1CCC=N1

> <INCHI_IDENTIFIER>
InChI=1S/C5H7NO2/c7-5(8)4-2-1-3-6-4/h3-4H,1-2H2,(H,7,8)/t4-/m0/s1

> <INCHI_KEY>
DWAKNKKXGALPNW-BYPYZUCNSA-N

> <FORMULA>
C5H7NO2

> <MOLECULAR_WEIGHT>
113.1146

> <EXACT_MASS>
113.047678473

> <JCHEM_ACCEPTOR_COUNT>
3

> <JCHEM_ATOM_COUNT>
15

> <JCHEM_AVERAGE_POLARIZABILITY>
10.721633827168107

> <JCHEM_BIOAVAILABILITY>
1

> <JCHEM_DONOR_COUNT>
1

> <JCHEM_FORMAL_CHARGE>
0

> <JCHEM_GHOSE_FILTER>
0

> <JCHEM_IUPAC>
(2S)-3,4-dihydro-2H-pyrrole-2-carboxylic acid

> <ALOGPS_LOGP>
-0.01

> <JCHEM_LOGP>
-2.279439073313947

> <ALOGPS_LOGS>
-0.94

> <JCHEM_MDDR_LIKE_RULE>
0

> <JCHEM_NUMBER_OF_RINGS>
1

> <JCHEM_PHYSIOLOGICAL_CHARGE>
-1

> <JCHEM_PKA_STRONGEST_ACIDIC>
1.8219556318598356

> <JCHEM_PKA_STRONGEST_BASIC>
6.073045352102196

> <JCHEM_POLAR_SURFACE_AREA>
49.66

> <JCHEM_REFRACTIVITY>
27.397299999999998

> <JCHEM_ROTATABLE_BOND_COUNT>
1

> <JCHEM_RULE_OF_FIVE>
1

> <ALOGPS_SOLUBILITY>
1.31e+01 g/l

> <JCHEM_TRADITIONAL_IUPAC>
L-1-pyrroline-5-carboxylate

> <JCHEM_VEBER_RULE>
0

$$$$

Download
3D-SDF for HMDB0001301 (1-Pyrroline-5-carboxylic acid)
440162
  -OEChem-10101913173D

 15 15  0     1  0  0  0  0  0999 V2000
   -2.1751    1.0357    0.1625 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.8135   -1.1153   -0.4996 O   0  0  0  0  0  0  0  0  0  0  0  0
    0.6969   -1.1177    0.3389 N   0  0  0  0  0  0  0  0  0  0  0  0
    0.7224    1.1997   -0.2633 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.0374    0.1492    0.5292 C   0  0  1  0  0  0  0  0  0  0  0  0
    2.1432    0.6910   -0.2187 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.8790   -0.7667   -0.0545 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.4157   -0.0760    0.0054 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.6236    2.2020    0.1651 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.3713    1.2505   -1.3020 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.0827    0.3778    1.6001 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.7047    1.0549    0.6459 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.6980    0.8922   -1.1373 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.6807   -1.4962   -0.2383 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.0861    0.9083   -0.1781 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  8  1  0  0  0  0
  1 15  1  0  0  0  0
  2  8  2  0  0  0  0
  3  5  1  0  0  0  0
  3  7  2  0  0  0  0
  4  5  1  0  0  0  0
  4  6  1  0  0  0  0
  4  9  1  0  0  0  0
  4 10  1  0  0  0  0
  5  8  1  0  0  0  0
  5 11  1  0  0  0  0
  6  7  1  0  0  0  0
  6 12  1  0  0  0  0
  6 13  1  0  0  0  0
  7 14  1  0  0  0  0
M  END
> <PUBCHEM_COMPOUND_CID>
440162

> <PUBCHEM_CONFORMER_RMSD>
0.4

> <PUBCHEM_CONFORMER_DIVERSEORDER>
1
4
3
2

> <PUBCHEM_MMFF94_PARTIAL_CHARGES>
9
1 -0.65
14 0.06
15 0.5
2 -0.57
3 -0.7
5 0.31
6 0.06
7 0.33
8 0.66

> <PUBCHEM_EFFECTIVE_ROTOR_COUNT>
1.6

> <PUBCHEM_PHARMACOPHORE_FEATURES>
5
1 1 acceptor
1 2 acceptor
1 3 acceptor
3 1 2 8 anion
5 3 4 5 6 7 rings

> <PUBCHEM_HEAVY_ATOM_COUNT>
8

> <PUBCHEM_ATOM_DEF_STEREO_COUNT>
1

> <PUBCHEM_ATOM_UDEF_STEREO_COUNT>
0

> <PUBCHEM_BOND_DEF_STEREO_COUNT>
0

> <PUBCHEM_BOND_UDEF_STEREO_COUNT>
0

> <PUBCHEM_ISOTOPIC_ATOM_COUNT>
0

> <PUBCHEM_COMPONENT_COUNT>
1

> <PUBCHEM_CACTVS_TAUTO_COUNT>
1

> <PUBCHEM_CONFORMER_ID>
0006B76200000001

> <PUBCHEM_MMFF94_ENERGY>
9.8895

> <PUBCHEM_FEATURE_SELFOVERLAP>
25.437

> <PUBCHEM_SHAPE_FINGERPRINT>
12897270 3 18267018547410328055
18185500 45 18411417280526315802
20096714 4 18407763637752268248
21040471 1 18335706108460616120
23552423 10 18335423452220420382
24536 1 17749392619280429905
29004967 10 18040725735752123360
5084963 1 17168982455606592718

> <PUBCHEM_SHAPE_MULTIPOLES>
147.92
2.89
1.29
0.68
0.41
0.01
-0.01
0.13
0.26
-0.03
0.06
0.01
-0.02
-0.22

> <PUBCHEM_SHAPE_SELFOVERLAP>
299.469

> <PUBCHEM_SHAPE_VOLUME>
87.8

> <PUBCHEM_COORDINATE_TYPE>
2
5
10

$$$$

Download
PDB for HMDB0001301 (1-Pyrroline-5-carboxylic acid)
HEADER    PROTEIN                                 14-SEP-20   NONE
TITLE     NULL                                                        
COMPND    NULL                                                        
SOURCE    NULL                                                        
KEYWDS    NULL                                                        
EXPDTA    NULL                                                        
AUTHOR    Marvin                                                      
REVDAT   1   14-SEP-20         0                                  
HETATM    1  C   UNK     0       1.248   0.338   0.000  0.00  0.00           C+0
HETATM    2  O   UNK     0       2.583  -0.430   0.000  0.00  0.00           O+0
HETATM    3  O   UNK     0       1.248   1.877   0.000  0.00  0.00           O+0
HETATM    4  N   UNK     0      -1.337   0.493   0.000  0.00  0.00           N+0
HETATM    5  C   UNK     0      -2.583  -0.412   0.000  0.00  0.00           C+0
HETATM    6  C   UNK     0      -2.107  -1.877   0.000  0.00  0.00           C+0
HETATM    7  C   UNK     0      -0.567  -1.877   0.000  0.00  0.00           C+0
HETATM    8  C   UNK     0      -0.091  -0.412   0.000  0.00  0.00           C+0
CONECT    1    2    3    8                                                  
CONECT    2    1                                                            
CONECT    3    1                                                            
CONECT    4    5    8                                                       
CONECT    5    6    4                                                       
CONECT    6    5    7                                                       
CONECT    7    6    8                                                       
CONECT    8    7    4    1                                                  
MASTER        0    0    0    0    0    0    0    0    8    0   16    0
END

Download
3D PDB for HMDB0001301 (1-Pyrroline-5-carboxylic acid)
COMPND    https://cactus.nci.nih.gov/chemical/structure/OC(=O)%5BC@@H%5D1CCC=N1/file?fo... 
AUTHOR    GENERATED BY OPEN BABEL 3.1.0
ATOM      1  O   PRO A   1       2.363  -0.736   0.274  1.00  0.00           O  
ATOM      2  C   PRO A   1       1.346   0.099   0.011  1.00  0.00           C  
ATOM      3  OXT PRO A   1       1.533   1.085  -0.662  1.00  0.00           O  
ATOM      4  CA  PRO A   1      -0.029  -0.193   0.554  1.00  0.00           C  
ATOM      5  HA  PRO A   1       0.039  -0.561   1.578  1.00  0.00           H  
ATOM      6  CB  PRO A   1      -0.753  -1.225  -0.342  1.00  0.00           C  
ATOM      7  CG  PRO A   1      -2.204  -0.687  -0.291  1.00  0.00           C  
ATOM      8  CD  PRO A   1      -2.033   0.776   0.052  1.00  0.00           C  
ATOM      9  N   PRO A   1      -0.864   1.013   0.502  1.00  0.00           N  
ATOM     10  H   PRO A   1       3.226  -0.507  -0.096  1.00  0.00           H  
ATOM     11  HB1 PRO A   1      -0.362  -1.206  -1.359  1.00  0.00           H  
ATOM     12  HB2 PRO A   1      -0.690  -2.227   0.083  1.00  0.00           H  
ATOM     13  HG1 PRO A   1      -2.688  -0.798  -1.262  1.00  0.00           H  
ATOM     14  HG2 PRO A   1      -2.775  -1.198   0.483  1.00  0.00           H  
ATOM     15  HD  PRO A   1      -2.803   1.525  -0.067  1.00  0.00           H  
CONECT    1    2   10                                                 
CONECT    2    1    3    3    4                                       
CONECT    3    2    2                                                 
CONECT    4    2    5    6    9                                       
CONECT    5    4                                                      
CONECT    6    4    7   11   12                                       
CONECT    7    6    8   13   14                                       
CONECT    8    7    9    9   15                                       
CONECT    9    8    8    4                                            
CONECT   10    1                                                      
CONECT   11    6                                                      
CONECT   12    6                                                      
CONECT   13    7                                                      
CONECT   14    7                                                      
CONECT   15    8                                                      
MASTER        0    0    0    0    0    0    0    0   15    0   15    0
END

Download
SMILES for HMDB0001301 (1-Pyrroline-5-carboxylic acid)
OC(=O)[C@@H]1CCC=N1
Download
INCHI for HMDB0001301 (1-Pyrroline-5-carboxylic acid)
InChI=1S/C5H7NO2/c7-5(8)4-2-1-3-6-4/h3-4H,1-2H2,(H,7,8)/t4-/m0/s1
Download
View in JSmolView Stereo Labels
Synonyms
ValueSource
1-Pyrroline-5-carboxylateChEBI, KEGG
L-1-Pyrroline-5-carboxylateChEBI, KEGG
1-Pyrroline-5-carboxylic acidGenerator
(S)-1-Pyrroline-5-carboxylic acidGenerator
L-1-Pyrroline-5-carboxylic acidGenerator
delta-1-Pyrroline-5-carboxylateMeSH
delta-1-Pyrroline-5-carboxylate, (+-)-isomerMeSH
delta-1-Pyrroline-5-carboxylic acidMeSH
delta-1-Pyrroline-5-carboxylate, 14C-labeled, (+-)-isomerMeSH
delta(1)Pyrroline-5-carboxylateMeSH
Pyrroline-5-carboxylateMeSH
(2S)-3,4-Dihydro-2H-pyrrole-2-carboxylic acidHMDB
(S)-1-Pyrroline-5-carboxylateHMDB
3,4-Dihydro-2H-pyrrole-2-carboxylic acidHMDB
delta1-Pyrroline-5-carboxylic acidHMDB
Δ1-Pyrroline-5-carboxylic acidHMDB
Chemical FormulaC5H7NO2
Average Molecular Weight113.1146
Monoisotopic Molecular Weight113.047678473
IUPAC Name(2S)-3,4-dihydro-2H-pyrrole-2-carboxylic acid
Traditional NameL-1-pyrroline-5-carboxylate
CAS Registry Number64199-88-8
SMILES
OC(=O)[C@@H]1CCC=N1
InChI Identifier
InChI=1S/C5H7NO2/c7-5(8)4-2-1-3-6-4/h3-4H,1-2H2,(H,7,8)/t4-/m0/s1
InChI KeyDWAKNKKXGALPNW-BYPYZUCNSA-N
Chemical Taxonomy
Description 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
    • 

  • Pyrroline carboxylic acid or derivatives
    • 

  • Pyrroline
    • 

  • Carboxylic acid
    • 

  • Monocarboxylic acid or derivatives
    • 

  • Propargyl-type 1,3-dipolar organic compound
    • 

  • Organic 1,3-dipolar compound
    • 

  • Organoheterocyclic compound
    • 

  • Azacycle
    • 

  • Organopnictogen compound
    • 

  • Organic oxygen compound
    • 

  • Organooxygen compound
    • 

  • Organonitrogen compound
    • 

  • Organic oxide
    • 

  • Carbonyl group
    • 

  • Imine
    • 

  • Organic nitrogen compound
    • 

  • Hydrocarbon derivative
    • 

  • Aliphatic heteromonocyclic compound
Molecular FrameworkAliphatic heteromonocyclic compounds
External Descriptors
Ontology
Disposition
Source:
Biological location:
Process
Naturally occurring process:
Role
Indirect biological role:
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 g/LALOGPS
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 ŲChemAxon
Rotatable Bond Count1ChemAxon
Refractivity27.4 m³·mol⁻¹ChemAxon
Polarizability10.72 ųChemAxon
Number of Rings1ChemAxon
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Spectra
GC-MS
Spectrum TypeDescriptionSplash KeyDeposition DateView
MSMass Spectrum (Electron Ionization)splash10-016u-9000000000-adea27ab28610a5698932021-09-05View Spectrum
LC-MS/MS
Spectrum TypeDescriptionSplash KeyDeposition DateView
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum -  10V, Positivesplash10-03dj-9700000000-f2bc2275638f96dc74ac2015-09-15View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum -  20V, Positivesplash10-014i-9100000000-72dff6639c24448bec9c2015-09-15View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum -  40V, Positivesplash10-014l-9000000000-28eb1358005b28459deb2015-09-15View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum -  10V, Negativesplash10-03di-5900000000-0720aa91caee03b9364e2015-09-15View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum -  20V, Negativesplash10-02t9-9400000000-cfcc87863f675fb126912015-09-15View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum -  40V, Negativesplash10-00kf-9000000000-a15b91c200295166bf9e2015-09-15View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum -  10V, Positivesplash10-014i-9000000000-e0673bc7e6a6226fb72e2021-09-06View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum -  40V, Positivesplash10-014i-9000000000-4f0cf32a2f9df52b4ad32021-09-06View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum -  10V, Negativesplash10-03di-2900000000-043a05a728838fd3b2fe2021-09-06View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum -  20V, Negativesplash10-014i-9200000000-91f194ef0ca23d3de3182021-09-06View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum -  40V, Negativesplash10-00kf-9000000000-f29d6251972eea5ba6132021-09-06View Spectrum
Biological Properties
Cellular Locations
  • Mitochondria
Biospecimen Locations
  • Feces
Tissue Locations
  • Intestine
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
 
Normal		  details
Abnormal Concentrations
Not Available
Associated Disorders and Diseases
Disease ReferencesNone
Associated OMIM IDsNone
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FooDB IDFDB030153  
KNApSAcK IDC00007606  
Chemspider ID389153  
KEGG Compound IDC03912  
BioCyc IDL-DELTA1-PYRROLINE_5-CARBOXYLATE  
BiGG IDNot Available
Wikipedia Link1-Pyrroline-5-carboxylic acid  
METLIN IDNot Available
PubChem Compound440162  
PDB IDNot Available
ChEBI ID371  
Food Biomarker OntologyNot Available
VMH IDNot Available
MarkerDB IDNot Available
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. [PubMed:533224  ] 
  2. 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. [PubMed:3392037  ] 
  3. Wakabayashi Y: Tissue-selective expression of enzymes of arginine synthesis. Curr Opin Clin Nutr Metab Care. 1998 Jul;1(4):335-9. [PubMed:10565370  ] 
  4. 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  ] 
  5. Fleming GA, Hagedorn CH, Granger AS, Phang JM: Pyrroline-5-carboxylate in human plasma. Metabolism. 1984 Aug;33(8):739-42. [PubMed:6748947  ] 
  6. 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. [PubMed:11510941  ] 
  7. Deuschle K, Funck D, Forlani G, Stransky H, Biehl A, Leister D, van der Graaff E, Kunze R, Frommer WB: The role of [Delta]1-pyrroline-5-carboxylate dehydrogenase in proline degradation. Plant Cell. 2004 Dec;16(12):3413-25. doi: 10.1105/tpc.104.023622. Epub 2004 Nov 17. [PubMed:15548746  ] 
Enzymes
  Enzyme Details
1. Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial
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
  Enzyme Details
2. Pyrroline-5-carboxylate reductase 1, mitochondrial
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
  Enzyme Details
3. Pyrroline-5-carboxylate reductase 2
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
  Enzyme Details
4. Proline dehydrogenase 1, mitochondrial
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
  Enzyme Details
5. Pyrroline-5-carboxylate reductase 3
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
  Enzyme Details
6. Probable proline dehydrogenase 2
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