Peroxisomal bifunctional enzyme

Details

Name
Peroxisomal bifunctional enzyme
Synonyms
  • ECHD
  • PBE
Gene Name
EHHADH
Organism
Humans
Amino acid sequence
>lcl|BSEQ0037049|Peroxisomal bifunctional enzyme
MAEYTRLHNALALIRLRNPPVNAISTTLLRDIKEGLQKAVIDHTIKAIVICGAEGKFSAG
ADIRGFSAPRTFGLTLGHVVDEIQRNEKPVVAAIQGMAFGGGLELALGCHYRIAHAEAQV
GLPEVTLGLLPGARGTQLLPRLTGVPAALDLITSGRRILADEALKLGILDKVVNSDPVEE
AIRFAQRVSDQPLESRRLCNKPIQSLPNMDSIFSEALLKMRRQHPGCLAQEACVRAVQAA
VQYPYEVGIKKEEELFLYLLQSGQARALQYAFFAERKANKWSTPSGASWKTASARPVSSV
GVVGLGTMGRGIVISFARARIPVIAVDSDKNQLATANKMITSVLEKEASKMQQSGHPWSG
PKPRLTSSVKELGGVDLVIEAVFEEMSLKKQVFAELSAVCKPEAFLCTNTSALDVDEIAS
STDRPHLVIGTHFFSPAHVMKLLEVIPSQYSSPTTIATVMNLSKKIKKIGVVVGNCFGFV
GNRMLNPYYNQAYFLLEEGSKPEEVDQVLEEFGFKMGPFRVSDLAGLDVGWKSRKGQGLT
GPTLLPGTPARKRGNRRYCPIPDVLCELGRFGQKTGKGWYQYDKPLGRIHKPDPWLSKFL
SRYRKTHHIEPRTISQDEILERCLYSLINEAFRILGEGIAASPEHIDVVYLHGYGWPRHK
GGPMFYASTVGLPTVLEKLQKYYRQNPDIPQLEPSDYLKKLASQGNPPLKEWQSLAGSPS
SKL
Number of residues
723
Molecular Weight
79494.18
Theoretical pI
9.49
GO Classification
Functions
3-hydroxyacyl-CoA dehydrogenase activity / dodecenoyl-CoA delta-isomerase activity / enoyl-CoA hydratase activity / enzyme binding / receptor binding
Processes
fatty acid beta-oxidation / internal protein amino acid acetylation
Components
cytosol / mitochondrion / peroxisome
General Function
Receptor binding
Specific Function
Not Available
Pfam Domain Function
Transmembrane Regions
Not Available
Cellular Location
Peroxisome
Gene sequence
>lcl|BSEQ0010409|Peroxisomal bifunctional enzyme (EHHADH)
ATGGCTTTCGGAGGGGGACTAGAGCTGGCCCTGGGCTGTCACTATAGGATTGCCCACGCA
GAGGCTCAAGTTGGCTTACCAGAAGTTACACTGGGACTTCTCCCTGGTGCAAGAGGAACC
CAGCTTCTCCCCAGACTCACTGGAGTTCCTGCTGCACTTGACTTAATTACCTCAGGAAGA
CGTATTTTAGCAGATGAAGCACTCAAGCTGGGCATTCTAGATAAAGTTGTAAACTCAGAC
CCGGTTGAAGAAGCAATCAGATTTGCTCAGAGAGTTTCAGATCAACCTCTAGAATCCCGT
AGACTCTGCAACAAGCCAATTCAGAGCTTGCCCAACATGGACAGCATTTTTAGTGAGGCC
CTCTTGAAGATGCGGAGGCAGCACCCTGGGTGTCTTGCACAGGAGGCTTGTGTCCGTGCA
GTCCAGGCTGCTGTGCAGTATCCCTATGAAGTGGGCATCAAGAAGGAGGAGGAGCTGTTT
CTATATCTTTTGCAATCAGGGCAGGCTAGAGCCCTGCAATATGCTTTCTTCGCTGAAAGG
AAAGCAAATAAGTGGTCAACTCCCTCCGGAGCATCGTGGAAAACAGCATCAGCGCGGCCT
GTCTCCTCAGTTGGTGTTGTTGGCTTGGGAACAATGGGCCGAGGCATTGTCATTTCTTTT
GCAAGGGCCAGGATTCCTGTGATTGCTGTAGACTCGGACAAAAACCAGCTAGCAACTGCA
AACAAGATGATAACCTCTGTCTTGGAAAAAGAAGCCTCCAAAATGCAACAGAGCGGCCAC
CCTTGGTCAGGACCAAAACCCAGGTTAACTTCATCTGTGAAGGAGCTTGGTGGTGTAGAT
TTAGTCATTGAAGCAGTATTTGAGGAAATGAGCCTGAAGAAGCAGGTCTTTGCTGAACTC
TCAGCTGTGTGCAAACCAGAAGCATTTTTGTGCACTAATACTTCAGCCCTGGATGTTGAT
GAGATTGCTTCTTCCACTGATCGTCCTCACTTGGTCATTGGCACCCACTTCTTTTCGCCA
GCTCATGTCATGAAGTTGTTAGAGGTTATTCCCAGCCAATACTCTTCCCCCACTACCATT
GCCACTGTTATGAACTTATCAAAAAAGATTAAAAAGATTGGAGTCGTTGTAGGCAACTGT
TTTGGATTTGTGGGGAATCGAATGTTGAATCCTTACTACAATCAGGCATATTTCTTGTTA
GAAGAAGGCAGCAAACCAGAGGAGGTAGATCAGGTGCTGGAAGAGTTTGGTTTTAAAATG
GGACCTTTTAGAGTGTCTGATCTTGCTGGGTTGGATGTGGGCTGGAAATCTAGAAAGGGG
CAAGGTCTTACTGGACCTACATTGCTTCCAGGAACTCCTGCCCGAAAAAGGGGTAATAGG
AGGTACTGCCCAATTCCTGATGTGCTCTGTGAATTAGGACGATTTGGCCAGAAGACAGGT
AAGGGTTGGTATCAATATGACAAGCCATTGGGTAGGATTCACAAACCTGATCCCTGGCTT
TCCAAATTCCTATCACGGTATAGAAAAACCCATCACATTGAACCACGTACCATTAGCCAG
GATGAGATCCTTGAACGCTGCTTATATTCACTTATCAATGAAGCATTCCGTATCTTGGGA
GAAGGGATAGCTGCTAGCCCAGAGCACATTGATGTTGTCTATTTACATGGATATGGATGG
CCAAGGCACAAGGGCGGGCCCATGTTCTATGCTTCCACAGTTGGGTTGCCCACAGTTCTA
GAGAAATTGCAGAAATATTACAGGCAGAACCCTGATATTCCCCAACTGGAGCCAAGTGAC
TATCTAAAAAAACTGGCTTCTCAGGGAAACCCTCCCCTGAAAGAATGGCAAAGCTTGGCA
GGCTCCCCTAGCAGTAAATTGTGA
Chromosome Location
3
Locus
3q26.3-q28
External Identifiers
ResourceLink
UniProtKB IDQ08426
UniProtKB Entry NameECHP_HUMAN
GenBank Protein ID452045
GenBank Gene IDL07077
GenAtlas IDEHHADH
HGNC IDHGNC:3247
General References
  1. Hoefler G, Forstner M, McGuinness MC, Hulla W, Hiden M, Krisper P, Kenner L, Ried T, Lengauer C, Zechner R, et al.: cDNA cloning of the human peroxisomal enoyl-CoA hydratase: 3-hydroxyacyl-CoA dehydrogenase bifunctional enzyme and localization to chromosome 3q26.3-3q28: a free left Alu Arm is inserted in the 3' noncoding region. Genomics. 1994 Jan 1;19(1):60-7. [Article]
  2. Ota T, Suzuki Y, Nishikawa T, Otsuki T, Sugiyama T, Irie R, Wakamatsu A, Hayashi K, Sato H, Nagai K, Kimura K, Makita H, Sekine M, Obayashi M, Nishi T, Shibahara T, Tanaka T, Ishii S, Yamamoto J, Saito K, Kawai Y, Isono Y, Nakamura Y, Nagahari K, Murakami K, Yasuda T, Iwayanagi T, Wagatsuma M, Shiratori A, Sudo H, Hosoiri T, Kaku Y, Kodaira H, Kondo H, Sugawara M, Takahashi M, Kanda K, Yokoi T, Furuya T, Kikkawa E, Omura Y, Abe K, Kamihara K, Katsuta N, Sato K, Tanikawa M, Yamazaki M, Ninomiya K, Ishibashi T, Yamashita H, Murakawa K, Fujimori K, Tanai H, Kimata M, Watanabe M, Hiraoka S, Chiba Y, Ishida S, Ono Y, Takiguchi S, Watanabe S, Yosida M, Hotuta T, Kusano J, Kanehori K, Takahashi-Fujii A, Hara H, Tanase TO, Nomura Y, Togiya S, Komai F, Hara R, Takeuchi K, Arita M, Imose N, Musashino K, Yuuki H, Oshima A, Sasaki N, Aotsuka S, Yoshikawa Y, Matsunawa H, Ichihara T, Shiohata N, Sano S, Moriya S, Momiyama H, Satoh N, Takami S, Terashima Y, Suzuki O, Nakagawa S, Senoh A, Mizoguchi H, Goto Y, Shimizu F, Wakebe H, Hishigaki H, Watanabe T, Sugiyama A, Takemoto M, Kawakami B, Yamazaki M, Watanabe K, Kumagai A, Itakura S, Fukuzumi Y, Fujimori Y, Komiyama M, Tashiro H, Tanigami A, Fujiwara T, Ono T, Yamada K, Fujii Y, Ozaki K, Hirao M, Ohmori Y, Kawabata A, Hikiji T, Kobatake N, Inagaki H, Ikema Y, Okamoto S, Okitani R, Kawakami T, Noguchi S, Itoh T, Shigeta K, Senba T, Matsumura K, Nakajima Y, Mizuno T, Morinaga M, Sasaki M, Togashi T, Oyama M, Hata H, Watanabe M, Komatsu T, Mizushima-Sugano J, Satoh T, Shirai Y, Takahashi Y, Nakagawa K, Okumura K, Nagase T, Nomura N, Kikuchi H, Masuho Y, Yamashita R, Nakai K, Yada T, Nakamura Y, Ohara O, Isogai T, Sugano S: Complete sequencing and characterization of 21,243 full-length human cDNAs. Nat Genet. 2004 Jan;36(1):40-5. Epub 2003 Dec 21. [Article]
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  4. 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. [Article]
  5. Chen GL, Balfe A, Erwa W, Hoefler G, Gaertner J, Aikawa J, Chen WW: Import of human bifunctional enzyme into peroxisomes of human hepatoma cells in vitro. Biochem Biophys Res Commun. 1991 Aug 15;178(3):1084-91. [Article]
  6. Chen WW, Watkins PA, Osumi T, Hashimoto T, Moser HW: Peroxisomal beta-oxidation enzyme proteins in adrenoleukodystrophy: distinction between X-linked adrenoleukodystrophy and neonatal adrenoleukodystrophy. Proc Natl Acad Sci U S A. 1987 Mar;84(5):1425-8. [Article]
  7. Choudhary C, Kumar C, Gnad F, Nielsen ML, Rehman M, Walther TC, Olsen JV, Mann M: Lysine acetylation targets protein complexes and co-regulates major cellular functions. Science. 2009 Aug 14;325(5942):834-40. doi: 10.1126/science.1175371. Epub 2009 Jul 16. [Article]
  8. 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: Regulation of cellular metabolism by protein lysine acetylation. Science. 2010 Feb 19;327(5968):1000-4. doi: 10.1126/science.1179689. [Article]
  9. Burkard TR, Planyavsky M, Kaupe I, Breitwieser FP, Burckstummer T, Bennett KL, Superti-Furga G, Colinge J: Initial characterization of the human central proteome. BMC Syst Biol. 2011 Jan 26;5:17. doi: 10.1186/1752-0509-5-17. [Article]
  10. Bian Y, Song C, Cheng K, Dong M, Wang F, Huang J, Sun D, Wang L, Ye M, Zou H: An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome. J Proteomics. 2014 Jan 16;96:253-62. doi: 10.1016/j.jprot.2013.11.014. Epub 2013 Nov 22. [Article]
  11. Klootwijk ED, Reichold M, Helip-Wooley A, Tolaymat A, Broeker C, Robinette SL, Reinders J, Peindl D, Renner K, Eberhart K, Assmann N, Oefner PJ, Dettmer K, Sterner C, Schroeder J, Zorger N, Witzgall R, Reinhold SW, Stanescu HC, Bockenhauer D, Jaureguiberry G, Courtneidge H, Hall AM, Wijeyesekera AD, Holmes E, Nicholson JK, O'Brien K, Bernardini I, Krasnewich DM, Arcos-Burgos M, Izumi Y, Nonoguchi H, Jia Y, Reddy JK, Ilyas M, Unwin RJ, Gahl WA, Warth R, Kleta R: Mistargeting of peroxisomal EHHADH and inherited renal Fanconi's syndrome. N Engl J Med. 2014 Jan 9;370(2):129-38. doi: 10.1056/NEJMoa1307581. [Article]

Drug Relations

Drug Relations
DrugBank IDNameDrug groupPharmacological action?ActionsDetails
DB00157NADHapproved, nutraceuticalunknownDetails