ALDH4A1 - Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial - human protein (Function)
 
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ALDH4A1 »  Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial   [ EC 1.2.1.88 ]  (P5C dehydrogenase)
 
Protein also known as:  L-glutamate gamma-semialdehyde dehydrogenase.
Gene name:  ALDH4A1
Family name: Aldehyde dehydrogenase
Entry whose protein(s) existence is based on evidence at protein level
extend overview
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GENE REF ISO

Function

 show evidences
Overview 
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.  
1
  • CuratedUniProtKB
GO molecular function 
Aldehyde dehydrogenase (NAD) activitydefinition[GO:0004029]  
1
  • TASPINC
Electron carrier activitydefinition[GO:0009055]  
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  • TASUniProtKB
Identical protein bindingdefinition[GO:0042802]  
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  • IPIIntAct
GO biological process 
4-hydroxyproline catabolic processdefinition[GO:0019470]  
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  • TASBHF-UCL
Proline biosynthetic processdefinition[GO:0006561] silver  
  • IEAInterPro 2 GO
Proline catabolic process to glutamatedefinition[GO:0010133]  
  • IEAUniPathway
Proline metabolic processdefinition[GO:0006560]  
1
  • TASPINC
Enzymatic activity 
This protein acts as an enzyme. It is known to catalyze the following reaction
EC 1.2.1.88: L-glutamate 5-semialdehyde + NAD(+) + H(2)O L-glutamate + NADH.  
1
  • CuratedUniProtKB
Pathways 
This protein is involved in the following pathway
According to KEGG, this protein belongs to the following pathways:
Alanine, aspartate and glutamate metabolism  hsa00250+8659  
Arginine and proline metabolism  hsa00330+8659  
Metabolic pathways  hsa01100+8659  
According to Reactome, this protein belongs to the following pathway:
Metabolism  REACT_111217  
 
More information is available from:
 

Biophysicochemical properties

Kinetic parameters
KM 32 uM for L-pyrroline-5-carboxylate
KM 100 uM for NAD

Keywords

Biological process 
Proline metabolism  definition   [KW-0642]
Molecular function 
Oxidoreductase  definition   [KW-0560]
Technical term 
Reference proteome  definition   [KW-1185]
 

Further external links

Enzyme and pathway databases
SABIO-RK: P30038
Other
GeneWiki: Aldehyde_dehydrogenase_4_family,_member_A1
GenomeRNAi: 8659
PRO: PR:P30038