UniProt Accession

 AL4A1_HUMAN; P30038; A8K1Q7; B4DGE4; Q16882; Q53HU4; Q5JNV6; Q8IZ38; Q96IF0; Q9UDI6 

Genbank Protein ID

 U24267; U24266; AK222486; AK289972; AK294552; AL080251; AL954340; AL954340; AL080251; BX537160; CH471134; BC007581; BC023600 

Genbank Nucleotide ID

 AAC50501.1; AAC50500.1; BAD96206.1; BAF82661.1; BAG57755.1; CAI23417.1; CAI23417.1; CAI39493.1; CAI39493.1; EAW94858.1; AAH07581.1; AAH23600.1 

Protein Name

 Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial 

Protein Synonyms/Alias

 P5C dehydrogenase; Aldehyde dehydrogenase family 4 member A1 

Gene Name

 ALDH4A1 

Gene Synonyms/Alias

 ALDH4; P5CDH 

Created Date

 July 27, 2013 

Organism

 Homo sapiens (Human) 

NCBI Taxa ID

 9606 

Lysine Modification

Position	Peptide	Type	References
462	VYVYPDDKYKETLQL	acetylation	[1]
495	DVVQEATKVLRNAAG	ubiquitination	[2, 3]

Reference

 [1] Regulation of cellular metabolism by protein lysine acetylation.
 Zhao S, Xu W, Jiang W, Yu W, Lin Y, Zhang T, Yao J, Zhou L, Zeng Y, Li H, Li Y, Shi J, An W, Hancock SM, He F, Qin L, Chin J, Yang P, Chen X, Lei Q, Xiong Y, Guan KL.
 Science. 2010 Feb 19;327(5968):1000-4. [PMID: 20167786]
 [2] A proteome-wide, quantitative survey of in vivo ubiquitylation sites reveals widespread regulatory roles.
 Wagner SA, Beli P, Weinert BT, Nielsen ML, Cox J, Mann M, Choudhary C.
 Mol Cell Proteomics. 2011 Oct;10(10):M111.013284. [PMID: 21890473]
 [3] hCKSAAP_UbSite: improved prediction of human ubiquitination sites by exploiting amino acid pattern and properties.
 Chen Z, Zhou Y, Song J, Zhang Z.
 Biochim Biophys Acta. 2013 Aug;1834(8):1461-7. [PMID: 23603789] 

Functional Description

 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. 

Sequence Annotation

 NP_BIND 286 290 NAD (By similarity).
 ACT_SITE 314 314 Proton acceptor (By similarity).
 ACT_SITE 348 348 Nucleophile.
 BINDING 208 208 NAD (By similarity).
 BINDING 233 233 NAD (By similarity).
 BINDING 447 447 NAD (By similarity).
 BINDING 513 513 Substrate (By similarity).
 MOD_RES 99 99 N6-acetyllysine (By similarity).
 MOD_RES 114 114 N6-acetyllysine (By similarity).
 MOD_RES 402 402 N6-acetyllysine (By similarity).  

Keyword

 3D-structure; Acetylation; Alternative splicing; Complete proteome; Direct protein sequencing; Disease mutation; Mitochondrion; NAD; Oxidoreductase; Polymorphism; Proline metabolism; Reference proteome; Transit peptide. 

Sequence Source

 UniProt (SWISSPROT/TrEMBL); GenBank; EMBL 

Protein Length

 563 AA 

Protein Sequence

Gene Ontology

 GO:0005759; C:mitochondrial matrix; TAS:Reactome.
 GO:0003842; F:1-pyrroline-5-carboxylate dehydrogenase activity; EXP:Reactome.
 GO:0004029; F:aldehyde dehydrogenase (NAD) activity; TAS:ProtInc.
 GO:0009055; F:electron carrier activity; TAS:UniProtKB.
 GO:0019470; P:4-hydroxyproline catabolic process; TAS:BHF-UCL.
 GO:0034641; P:cellular nitrogen compound metabolic process; TAS:Reactome.
 GO:0006561; P:proline biosynthetic process; IEA:InterPro.
 GO:0006562; P:proline catabolic process; TAS:Reactome.
 GO:0010133; P:proline catabolic process to glutamate; IEA:UniProtKB-UniPathway. 

Interpro

 IPR005931; 1-pyrroline-5-COlate_DH.
 IPR016161; Ald_DH/histidinol_DH.
 IPR016163; Ald_DH_C.
 IPR016160; Ald_DH_CS.
 IPR016162; Ald_DH_N.
 IPR015590; Aldehyde_DH_dom. 

Pfam

 PF00171; Aldedh 

SMART

  

PROSITE

 PS00070; ALDEHYDE_DEHYDR_CYS
 PS00687; ALDEHYDE_DEHYDR_GLU 

PRINTS