Proline dehydrogenase 1, mitochondrial
Details
- Name
- Proline dehydrogenase 1, mitochondrial
- Synonyms
- 1.5.5.2
- p53-induced gene 6 protein
- PIG6
- POX2
- Proline oxidase
- Proline oxidase 2
- Gene Name
- PRODH
- Organism
- Humans
- Amino acid sequence
>lcl|BSEQ0037191|Proline dehydrogenase 1, mitochondrial MALRRALPALRPCIPRFVQLSTAPASREQPAAGPAAVPGGGSATAVRPPVPAVDFGNAQE AYRSRRTWELARSLLVLRLCAWPALLARHEQLLYVSRKLLGQRLFNKLMKMTFYGHFVAG EDQESIQPLLRHYRAFGVSAILDYGVEEDLSPEEAEHKEMESCTSAAERDGSGTNKRDKQ YQAHRAFGDRRNGVISARTYFYANEAKCDSHMETFLRCIEASGRVSDDGFIAIKLTALGR PQFLLQFSEVLAKWRCFFHQMAVEQGQAGLAAMDTKLEVAVLQESVAKLGIASRAEIEDW FTAETLGVSGTMDLLDWSSLIDSRTKLSKHLVVPNAQTGQLEPLLSRFTEEEELQMTRML QRMDVLAKKATEMGVRLMVDAEQTYFQPAISRLTLEMQRKFNVEKPLIFNTYQCYLKDAY DNVTLDVELARREGWCFGAKLVRGAYLAQERARAAEIGYEDPINPTYEATNAMYHRCLDY VLEELKHNAKAKVMVASHNEDTVRFALRRMEELGLHPADHQVYFGQLLGMCDQISFPLGQ AGYPVYKYVPYGPVMEVLPYLSRRALENSSLMKGTHRERQLLWLELLRRLRTGNLFHRPA
- Number of residues
- 600
- Molecular Weight
- 68001.405
- Theoretical pI
- 6.88
- GO Classification
- FunctionsFAD binding / proline dehydrogenase activityProcesses4-hydroxyproline catabolic process / cellular nitrogen compound metabolic process / intrinsic apoptotic signaling pathway in response to oxidative stress / proline catabolic process / proline catabolic process to glutamate / proline metabolic process / small molecule metabolic processComponentsmitochondrial inner membrane / mitochondrial matrix
- General Function
- Proline dehydrogenase activity
- Specific Function
- Converts proline to delta-1-pyrroline-5-carboxylate.
- Pfam Domain Function
- Pro_dh (PF01619)
- Transmembrane Regions
- Not Available
- Cellular Location
- Mitochondrion matrix
- Gene sequence
>lcl|BSEQ0019269|Proline dehydrogenase 1, mitochondrial (PRODH) ATGAAGATGACCTTCTATGGGCATTTTGTAGCCGGGGAGGACCAGGAGTCCATCCAGCCC CTGCTTCGGCACTACAGGGCCTTCGGTGTCAGCGCCATCCTGGACTATGGAGTGGAGGAG GACCTGAGCCCCGAGGAGGCAGAGCACAAGGAGATGGAGTCCTGCACCTCAGCTGCGGAG AGGGATGGCAGTGGCACGAATAAGCGGGACAAGCAATACCAGGCCCACCGGGCCTTCGGG GACCGCAGGAATGGTGTCATCAGTGCCCGCACCTACTTCTACGCCAATGAGGCCAAGTGC GACAGCCACATGGAGACATTCTTGCGCTGCATCGAAGCCTCAGGTAGAGTCAGCGATGAC GGCTTCATAGCCATTAAGCTCACAGCACTGGGGAGACCCCAGTTTCTGCTGCAGTTCTCA GAGGTGCTGGCCAAGTGGAGGTGCTTCTTTCACCAAATGGCTGTGGAGCAAGGGCAGGCG GGCCTGGCTGCCATGGACACCAAGCTGGAGGTGGCGGTGCTGCAGGAAAGTGTCGCAAAG TTGGGCATCGCATCCAGGGCTGAGATTGAGGACTGGTTCACGGCAGAGACCCTGGGAGTG TCTGGCACCATGGACCTGCTGGACTGGAGCAGCCTCATCGACAGCAGGACCAAGCTGTCC AAGCACTTGGTAGTCCCCAACGCACAGACAGGACAGCTGGAGCCCCTGCTGTCCCGGTTC ACTGAGGAGGAGGAGCTACAGATGACCAGGATGCTACAGCGGATGGATGTCCTGGCCAAG AAAGCCACAGAGATGGGCGTGCGGCTGATGGTGGATGCCGAGCAGACCTACTTCCAGCCG GCCATCAGCCGCCTGACGCTGGAGATGCAGCGGAAGTTCAATGTGGAGAAGCCGCTCATC TTCAACACATACCAGTGCTACCTCAAGGATGCCTATGACAATGTGACCCTGGACGTGGAG CTGGCTCGCCGTGAGGGCTGGTGTTTTGGGGCCAAGCTGGTGCGGGGCGCATACCTGGCC CAGGAGCGAGCCCGTGCGGCAGAGATCGGCTATGAGGACCCCATCAACCCCACGTACGAG GCCACCAACGCCATGTACCACAGGTGCCTGGACTACGTGTTGGAGGAGCTGAAGCACAAC GCCAAGGCCAAGGTGATGGTGGCCTCCCACAATGAGGACACAGTGCGCTTTGCACTGCGC AGGATGGAGGAGCTGGGCCTGCATCCTGCTGACCACCAGGTGTACTTTGGACAGCTGCTA GGCATGTGTGACCAGATCAGCTTCCCGCTGGGCCAGGCCGGCTACCCCGTGTACAAGTAC GTGCCCTATGGCCCCGTGATGGAGGTGCTGCCCTACTTGTCCCGCCGTGCCCTGGAGAAC AGCAGCCTCATGAAGGGCACCCATCGGGAGCGGCAGCTGCTGTGGCTGGAGCTCTTGAGG CGGCTCCGAACTGGCAACCTCTTCCATCGCCCTGCCTAG
- Chromosome Location
- 22
- Locus
- 22q11.21
- External Identifiers
Resource Link UniProtKB ID O43272 UniProtKB Entry Name PROD_HUMAN GenBank Protein ID 2677802 GenBank Gene ID U82381 GenAtlas ID PRODH HGNC ID HGNC:9453 - General References
- Campbell HD, Webb GC, Young IG: A human homologue of the Drosophila melanogaster sluggish-A (proline oxidase) gene maps to 22q11.2, and is a candidate gene for type-I hyperprolinaemia. Hum Genet. 1997 Nov;101(1):69-74. [Article]
- Gogos JA, Santha M, Takacs Z, Beck KD, Luine V, Lucas LR, Nadler JV, Karayiorgou M: The gene encoding proline dehydrogenase modulates sensorimotor gating in mice. Nat Genet. 1999 Apr;21(4):434-9. [Article]
- Bender HU, Almashanu S, Steel G, Hu CA, Lin WW, Willis A, Pulver A, Valle D: Functional consequences of PRODH missense mutations. Am J Hum Genet. 2005 Mar;76(3):409-20. Epub 2005 Jan 20. [Article]
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- Jacquet H, Raux G, Thibaut F, Hecketsweiler B, Houy E, Demilly C, Haouzir S, Allio G, Fouldrin G, Drouin V, Bou J, Petit M, Campion D, Frebourg T: PRODH mutations and hyperprolinemia in a subset of schizophrenic patients. Hum Mol Genet. 2002 Sep 15;11(19):2243-9. [Article]
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