Protein: Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial (P5C dehydrogenase) (EC 1.2.1.88) (Aldehyde dehydrogenase family 4 member A1) (L-glutamate gamma-semialdehyde dehydrogenase) (P30038)
Organism: Homo sapiens (Human)
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.
Subcellular location: Mitochondrion matrix.
Sequence: MLLPAPALRRALLSRPWTGAGLRWKHTSSLKVANEPVLAFTQGSPERDALQKALKDLKGRMEAIPCVVGDEEVWTSDVQYQVSPFNHGHKVAKFCYADKSLLNKAIEAALAARKEWDLKPIADRAQIFLKAADMLSGPRRAEILAKTMVGQGKTVIQAEIDAAAELIDFFRFNAKYAVELEGQQPISVPPSTNSTVYRGLEGFVAAISPFNFTAIGGNLAGAPALMGNVVLWKPSDTAMLASYAVYRILREAGLPPNIIQFVPADGPLFGDTVTSSEHLCGINFTGSVPTFKHLWKQVAQNLDRFHTFPRLAGECGGKNFHFVHRSADVESVVSGTLRSAFEYGGQKCSACSRLYVPHSLWPQIKGRLLEEHSRIKVGDPAEDFGTFFSAVIDAKSFARIKKWLEHARSSPSLTILAGGKCDDSVGYFVEPCIVESKDPQEPIMKEEIFGPVLSVYVYPDDKYKETLQLVDSTTSYGLTGAVFSQDKDVVQEATKVLRNAAGNFYINDKSTGSIVGQQPFGGARASGTNDKPGGPHYILRWTSPQVIKETHKPLGDWSYAYMQ
InterPro domains: - IPR016160: Aldehyde dehydrogenase, cysteine active site (conserved_site) [341-352]
- IPR029510: Aldehyde dehydrogenase, glutamic acid active site (conserved_site) [313-320]
- IPR015590: Aldehyde dehydrogenase domain (domain) [81-545]
- IPR016161: Aldehyde/histidinol dehydrogenase (homologous_superfamily) [30-547]
- IPR016162: Aldehyde dehydrogenase, N-terminal (homologous_superfamily) [35-549]
- IPR005931: Delta-1-pyrroline-5-carboxylate dehydrogenase (family) [32-563]
- IPR016163: Aldehyde dehydrogenase, C-terminal (homologous_superfamily) [316-514]
Interaction partners: - Proline dehydrogenase
- Proline dehydrogenase 1, mitochondrial
- Delta-1-pyrroline-5-carboxylate synthase
- Ornithine aminotransferase, mitochondrial
- Hydroxyproline dehydrogenase
- Pyrroline-5-carboxylate reductase 1, mitochondrial
- Pyrroline-5-carboxylate reductase 2
- Pyrroline-5-carboxylate reductase 3
- Glutamate dehydrogenase 1, mitochondrial
- Aspartate aminotransferase, cytoplasmic
Gene Ontology annotations:
Molecular Function (MF): GO:0003674 (molecular function), GO:0005488 (binding), GO:0003824 (catalytic activity), GO:0005515 (protein binding), GO:0016491 (oxidoreductase activity), GO:0042802 (identical protein binding), GO:0016903 (oxidoreductase activity, acting on the aldehyde or oxo group of donors), GO:0016620 (oxidoreductase activity, acting on the aldehyde or oxo group of donors, NAD or NADP as acceptor), GO:0004030 (aldehyde dehydrogenase [NAD(P)+] activity), GO:0004029 (aldehyde dehydrogenase (NAD+) activity)
Biological Process (BP): GO:0008150 (biological process), GO:0008152 (metabolic process), GO:0009987 (cellular process), GO:0044237 (cellular metabolic process), GO:0071704 (organic substance metabolic process), GO:0044281 (small molecule metabolic process), GO:0009056 (catabolic process), GO:0044238 (primary metabolic process), GO:0006807 (nitrogen compound metabolic process), GO:0006520 (amino acid metabolic process), GO:1901564 (organonitrogen compound metabolic process), GO:0044282 (small molecule catabolic process), GO:1901575 (organic substance catabolic process), GO:0006082 (organic acid metabolic process), GO:0044248 (cellular catabolic process), GO:0016054 (organic acid catabolic process), GO:1901565 (organonitrogen compound catabolic process), GO:0043436 (oxoacid metabolic process), GO:1901605 (alpha-amino acid metabolic process), GO:0009063 (amino acid catabolic process), GO:0019752 (carboxylic acid metabolic process), GO:1901606 (alpha-amino acid catabolic process), GO:0009064 (glutamine family amino acid metabolic process), GO:0046395 (carboxylic acid catabolic process), GO:0009065 (glutamine family amino acid catabolic process), GO:0006560 (proline metabolic process), GO:0006527 (arginine catabolic process), GO:0006525 (arginine metabolic process)
Cellular Component (CC): GO:0005575 (cellular component), GO:0110165 (cellular anatomical entity), GO:0005829 (cytosol), GO:0005622 (intracellular anatomical structure), GO:0043226 (organelle), GO:0005737 (cytoplasm), GO:0043229 (intracellular organelle), GO:0005739 (mitochondrion), GO:0043227 (membrane-bounded organelle), GO:0043231 (intracellular membrane-bounded organelle)