Proline dehydrogenase 1, mitochondrial

NameProline dehydrogenase 1, mitochondrial
Synonyms
  • 1.5.5.2
  • p53-induced gene 6 protein
  • PIG6
  • POX2
  • Proline oxidase
  • Proline oxidase 2
Gene NamePRODH
OrganismHuman
Amino acid sequence
>lcl|BSEQ0037191|Proline dehydrogenase 1, mitochondrial
MALRRALPALRPCIPRFVQLSTAPASREQPAAGPAAVPGGGSATAVRPPVPAVDFGNAQE
AYRSRRTWELARSLLVLRLCAWPALLARHEQLLYVSRKLLGQRLFNKLMKMTFYGHFVAG
EDQESIQPLLRHYRAFGVSAILDYGVEEDLSPEEAEHKEMESCTSAAERDGSGTNKRDKQ
YQAHRAFGDRRNGVISARTYFYANEAKCDSHMETFLRCIEASGRVSDDGFIAIKLTALGR
PQFLLQFSEVLAKWRCFFHQMAVEQGQAGLAAMDTKLEVAVLQESVAKLGIASRAEIEDW
FTAETLGVSGTMDLLDWSSLIDSRTKLSKHLVVPNAQTGQLEPLLSRFTEEEELQMTRML
QRMDVLAKKATEMGVRLMVDAEQTYFQPAISRLTLEMQRKFNVEKPLIFNTYQCYLKDAY
DNVTLDVELARREGWCFGAKLVRGAYLAQERARAAEIGYEDPINPTYEATNAMYHRCLDY
VLEELKHNAKAKVMVASHNEDTVRFALRRMEELGLHPADHQVYFGQLLGMCDQISFPLGQ
AGYPVYKYVPYGPVMEVLPYLSRRALENSSLMKGTHRERQLLWLELLRRLRTGNLFHRPA
Number of residues600
Molecular Weight68001.405
Theoretical pI6.88
GO Classification
Functions
  • FAD binding
  • proline dehydrogenase activity
Processes
  • 4-hydroxyproline catabolic process
  • proline catabolic process
  • proline catabolic process to glutamate
  • proline metabolic process
  • intrinsic apoptotic signaling pathway in response to oxidative stress
  • cellular nitrogen compound metabolic process
  • small molecule metabolic process
Components
  • mitochondrial inner membrane
  • mitochondrial matrix
General FunctionProline dehydrogenase activity
Specific FunctionConverts proline to delta-1-pyrroline-5-carboxylate.
Pfam Domain Function
Transmembrane RegionsNot Available
GenBank Protein ID2677802
UniProtKB IDO43272
UniProtKB Entry NamePROD_HUMAN
Cellular LocationMitochondrion 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
GenBank Gene IDU82381
GeneCard IDNot Available
GenAtlas IDPRODH
HGNC IDHGNC:9453
Chromosome Location22
Locus22q11.21
References
  1. 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. 9385373
  2. 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. 10192398
  3. 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. 15662599
  4. Dunham I, Shimizu N, Roe BA, Chissoe S, Hunt AR, Collins JE, Bruskiewich R, Beare DM, Clamp M, Smink LJ, Ainscough R, Almeida JP, Babbage A, Bagguley C, Bailey J, Barlow K, Bates KN, Beasley O, Bird CP, Blakey S, Bridgeman AM, Buck D, Burgess J, Burrill WD, O'Brien KP, et al.: The DNA sequence of human chromosome 22. Nature. 1999 Dec 2;402(6761):489-95. 10591208
  5. Gerhard DS, Wagner L, Feingold EA, Shenmen CM, Grouse LH, Schuler G, Klein SL, Old S, Rasooly R, Good P, Guyer M, Peck AM, Derge JG, Lipman D, Collins FS, Jang W, Sherry S, Feolo M, Misquitta L, Lee E, Rotmistrovsky K, Greenhut SF, Schaefer CF, Buetow K, Bonner TI, Haussler D, Kent J, Kiekhaus M, Furey T, Brent M, Prange C, Schreiber K, Shapiro N, Bhat NK, Hopkins RF, Hsie F, Driscoll T, Soares MB, Casavant TL, Scheetz TE, Brown-stein MJ, Usdin TB, Toshiyuki S, Carninci P, Piao Y, Dudekula DB, Ko MS, Kawakami K, Suzuki Y, Sugano S, Gruber CE, Smith MR, Simmons B, Moore T, Waterman R, Johnson SL, Ruan Y, Wei CL, Mathavan S, Gunaratne PH, Wu J, Garcia AM, Hulyk SW, Fuh E, Yuan Y, Sneed A, Kowis C, Hodgson A, Muzny DM, McPherson J, Gibbs RA, Fahey J, Helton E, Ketteman M, Madan A, Rodrigues S, Sanchez A, Whiting M, Madari A, Young AC, Wetherby KD, Granite SJ, Kwong PN, Brinkley CP, Pearson RL, Bouffard GG, Blakesly RW, Green ED, Dickson MC, Rodriguez AC, Grimwood J, Schmutz J, Myers RM, Butterfield YS, Griffith M, Griffith OL, Krzywinski MI, Liao N, Morin R, Palmquist D, Petrescu AS, Skalska U, Smailus DE, Stott JM, Schnerch A, Schein JE, Jones SJ, Holt RA, Baross A, Marra MA, Clifton S, Makowski KA, Bosak S, Malek J: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. 2004 Oct;14(10B):2121-7. 15489334
  6. Polyak K, Xia Y, Zweier JL, Kinzler KW, Vogelstein B: A model for p53-induced apoptosis. Nature. 1997 Sep 18;389(6648):300-5. 9305847
  7. 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. 12217952
  8. Liu H, Heath SC, Sobin C, Roos JL, Galke BL, Blundell ML, Lenane M, Robertson B, Wijsman EM, Rapoport JL, Gogos JA, Karayiorgou M: Genetic variation at the 22q11 PRODH2/DGCR6 locus presents an unusual pattern and increases susceptibility to schizophrenia. Proc Natl Acad Sci U S A. 2002 Mar 19;99(6):3717-22. Epub 2002 Mar 12. 11891283
  9. Sjoblom T, Jones S, Wood LD, Parsons DW, Lin J, Barber TD, Mandelker D, Leary RJ, Ptak J, Silliman N, Szabo S, Buckhaults P, Farrell C, Meeh P, Markowitz SD, Willis J, Dawson D, Willson JK, Gazdar AF, Hartigan J, Wu L, Liu C, Parmigiani G, Park BH, Bachman KE, Papadopoulos N, Vogelstein B, Kinzler KW, Velculescu VE: The consensus coding sequences of human breast and colorectal cancers. Science. 2006 Oct 13;314(5797):268-74. Epub 2006 Sep 7. 16959974
  10. Raux G, Bumsel E, Hecketsweiler B, van Amelsvoort T, Zinkstok J, Manouvrier-Hanu S, Fantini C, Breviere GM, Di Rosa G, Pustorino G, Vogels A, Swillen A, Legallic S, Bou J, Opolczynski G, Drouin-Garraud V, Lemarchand M, Philip N, Gerard-Desplanches A, Carlier M, Philippe A, Nolen MC, Heron D, Sarda P, Lacombe D, Coizet C, Alembik Y, Layet V, Afenjar A, Hannequin D, Demily C, Petit M, Thibaut F, Frebourg T, Campion D: Involvement of hyperprolinemia in cognitive and psychiatric features of the 22q11 deletion syndrome. Hum Mol Genet. 2007 Jan 1;16(1):83-91. Epub 2006 Nov 29. 17135275