| Identification |
|---|
| HMDB Protein ID
| CDBP00151 |
| Secondary Accession Numbers
| Not Available |
| Name
| Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial |
| Description
| Not Available |
| Synonyms
|
- Aldehyde dehydrogenase family 4 member A1
- P5C dehydrogenase
|
| Gene Name
| ALDH4A1 |
| Protein Type
| Enzyme |
| Biological Properties |
|---|
| 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.
|
| GO Classification
|
| Biological Process |
| cellular nitrogen compound metabolic process |
| 4-hydroxyproline catabolic process |
| proline biosynthetic process |
| proline catabolic process |
| proline catabolic process to glutamate |
| Cellular Component |
| mitochondrial matrix |
| Component |
| cell part |
| intracellular part |
| cytoplasmic part |
| mitochondrial part |
| mitochondrial matrix |
| Function |
| oxidoreductase activity |
| catalytic activity |
| oxidoreductase activity, acting on the ch-nh group of donors |
| oxidoreductase activity, acting on the ch-nh group of donors, nad or nadp as acceptor |
| 1-pyrroline-5-carboxylate dehydrogenase activity |
| Molecular Function |
| electron carrier activity |
| aldehyde dehydrogenase (NAD) activity |
| 1-pyrroline-5-carboxylate dehydrogenase activity |
| Process |
| metabolic process |
| cellular metabolic process |
| glutamine family amino acid metabolic process |
| proline metabolic process |
| proline biosynthetic process |
| oxidation reduction |
| cellular amino acid and derivative metabolic process |
| cellular amino acid metabolic process |
|
| Cellular Location
|
- Mitochondrion matrix
|
| Pathways
|
| Name | SMPDB/Pathwhiz | KEGG | | L-proline degradation into L-glutamate | Not Available | Not Available | | Alanine, aspartate and glutamate metabolism | Not Available |  | | Arginine and Proline Metabolism |    |  | | Prolidase Deficiency (PD) |    | Not Available | | Arginine: Glycine Amidinotransferase Deficiency (AGAT Deficiency) |    | Not Available |
|
| Gene Properties |
|---|
| Chromosome Location
| 1 |
| Locus
| 1p36 |
| SNPs
| ALDH4A1 |
| Gene Sequence
|
>1692 bp
ATGCTGCTGCCGGCGCCCGCGCTCCGCCGCGCCCTGCTGTCCCGCCCCTGGACCGGGGCC
GGCCTGCGGTGGAAGCACACCTCCTCCCTGAAGGTGGCCAACGAGCCCGTCTTAGCCTTC
ACGCAGGGCAGCCCTGAGCGAGATGCCCTGCAAAAGGCCTTGAAGGACCTGAAGGGCCGG
ATGGAAGCCATCCCATGCGTGGTGGGGGATGAGGAGGTGTGGACGTCGGACGTGCAGTAC
CAAGTGTCGCCTTTTAACCATGGACATAAGGTGGCCAAGTTCTGTTATGCAGACAAGAGC
CTGCTCAACAAAGCCATTGAGGCTGCCCTGGCTGCCCGGAAAGAGTGGGACCTGAAGCCT
ATTGCAGACCGGGCCCAGATCTTCCTGAAGGCGGCAGACATGCTGAGTGGGCCGCGCAGG
GCTGAGATCCTCGCCAAGACCATGGTGGGACAGGGTAAGACCGTGATCCAAGCGGAGATT
GACGCTGCAGCGGAACTCATCGACTTCTTCCGGTTCAATGCCAAGTATGCGGTGGAGCTG
GAGGGGCAGCAGCCCATCAGCGTGCCCCCGAGCACCAACAGCACGGTGTACCGGGGTCTG
GAGGGCTTCGTGGCGGCCATCTCGCCCTTTAACTTCACTGCAATCGGCGGCAACCTGGCG
GGGGCACCGGCCCTGATAGGCAACGTGGTCCTATGGAAGCCCAGTGACACTGCCATGCTG
GCCAGCTATGCTGTCTACCGCATCCTTCGGGAGGCTGGCCTGCCCCCCAACATCATCCAG
TTTGTGCCAGCTGATGGGCCCCTATTTGGGGACACTGTCACCAGCTCAGAGCACCTCTGT
GGCATCAACTTCACAGGCAGTGTGCCCACCTTCAAACACCTGTGGAAGCAGGTGGCCCAG
AACCTGGACCGGTTCCACACCTTCCCACGCCTGGCTGGAGAGTGCGGCGGAAAGAACTTC
CACTTCGTGCACCGCTCGGCCGACGTGGAGAGCGTGGTGAGCGGGACCCTCCGCTCAGCC
TTCGAGTACGGTGGCCAGAAGTGTTCCGCCTGCTCGCGTCTCTACGTGCCGCACTCGCTG
TGGCCGCAGATCAAAGGGCGGCTGCTGGAGGAGCACAGTCGGATCAGAGTGGGCGACCCT
GCAGAGGATTTTGGGACCTTCTTCTCTGCAGTGATTGATGCCAAGTCCTTTGCCCGTATC
AAGAAGTGGCTGGAGCACGCACGCTCCTCACCCAGCCTCACCATCCTGGCCGGGGGCAAG
TGTGATGACTCCGTGGGCTACTTTGTGGAGCCCTGCATCGTGGAGAGCAAGGACCCTCAG
GAGCCCATCATGAAGGAGGAGATCTTCGGGCCTGTACTGTCTGTGTACGTCTACCCGGAT
GACAAGTACAAGGAGACGCTGCAGCTGGTTGACAGCACCACCAGCTATGGCCTCACGGGG
GCAGTGTTCTCCCAGGATAAGGACGTCGTGCAGGAGGCCACAAAGGTGCTGAGGAATGCT
GCCGGCAACTTCTACATCAACGACAAGTCCACTGGCTCGATAGTGGGCCAGCAGCCCTTT
GGGGGGGCCCGAGCCTCTGGAACCAATGACAAGCCAGGGGGCCCACACTACATCCTGCGC
TGGACGTCGCCGCAGGTCATCAAGGAGACACATAAGCCCCTGGGGGACTGGAGCTACGCG
TACATGCAGTGA
|
| Protein Properties |
|---|
| Number of Residues
| 563 |
| Molecular Weight
| 55117.24 |
| Theoretical pI
| 6.797 |
| Pfam Domain Function
|
|
| Signals
|
Not Available
|
|
Transmembrane Regions
|
Not Available
|
| Protein Sequence
|
>Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial
MLLPAPALRRALLSRPWTGAGLRWKHTSSLKVANEPVLAFTQGSPERDALQKALKDLKGR
MEAIPCVVGDEEVWTSDVQYQVSPFNHGHKVAKFCYADKSLLNKAIEAALAARKEWDLKP
IADRAQIFLKAADMLSGPRRAEILAKTMVGQGKTVIQAEIDAAAELIDFFRFNAKYAVEL
EGQQPISVPPSTNSTVYRGLEGFVAAISPFNFTAIGGNLAGAPALMGNVVLWKPSDTAML
ASYAVYRILREAGLPPNIIQFVPADGPLFGDTVTSSEHLCGINFTGSVPTFKHLWKQVAQ
NLDRFHTFPRLAGECGGKNFHFVHRSADVESVVSGTLRSAFEYGGQKCSACSRLYVPHSL
WPQIKGRLLEEHSRIKVGDPAEDFGTFFSAVIDAKSFARIKKWLEHARSSPSLTILAGGK
CDDSVGYFVEPCIVESKDPQEPIMKEEIFGPVLSVYVYPDDKYKETLQLVDSTTSYGLTG
AVFSQDKDVVQEATKVLRNAAGNFYINDKSTGSIVGQQPFGGARASGTNDKPGGPHYILR
WTSPQVIKETHKPLGDWSYAYMQ
|
| External Links |
|---|
| GenBank ID Protein
| 62896531 |
| UniProtKB/Swiss-Prot ID
| P30038 |
| UniProtKB/Swiss-Prot Entry Name
| AL4A1_HUMAN |
| PDB IDs
|
|
| GenBank Gene ID
| AK222486 |
| GeneCard ID
| ALDH4A1 |
| GenAtlas ID
| ALDH4A1 |
| HGNC ID
| HGNC:406 |
| References |
|---|
| General References
| Not Available |