Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial
Details
- Name
- Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial
- Synonyms
- 1.1.1.35
- HAD
- HADHSC
- HCDH
- Medium and short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase
- SCHAD
- Short-chain 3-hydroxyacyl-CoA dehydrogenase
- Gene Name
- HADH
- Organism
- Humans
- Amino acid sequence
>lcl|BSEQ0037004|Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial MAFVTRQFMRSVSSSSTASASAKKIIVKHVTVIGGGLMGAGIAQVAAATGHTVVLVDQTE DILAKSKKGIEESLRKVAKKKFAENLKAGDEFVEKTLSTIATSTDAASVVHSTDLVVEAI VENLKVKNELFKRLDKFAAEHTIFASNTSSLQITSIANATTRQDRFAGLHFFNPVPVMKL VEVIKTPMTSQKTFESLVDFSKALGKHPVSCKDTPGFIVNRLLVPYLMEAIRLYERGDAS KEDIDTAMKLGAGYPMGPFELLDYVGLDTTKFIVDGWHEMDAENPLHQPSPSLNKLVAEN KFGKKTGEGFYKYK
- Number of residues
- 314
- Molecular Weight
- 34293.275
- Theoretical pI
- 9.41
- GO Classification
- Functions3-hydroxyacyl-CoA dehydrogenase activity / NAD+ bindingProcessescellular lipid metabolic process / fatty acid beta-oxidation / negative regulation of insulin secretion / response to activity / response to drug / response to insulin / small molecule metabolic processComponentscytoplasm / mitochondrial inner membrane / mitochondrial matrix / mitochondrion / nucleoplasm
- General Function
- Nad+ binding
- Specific Function
- Plays an essential role in the mitochondrial beta-oxidation of short chain fatty acids. Exerts it highest activity toward 3-hydroxybutyryl-CoA.
- Pfam Domain Function
- Transmembrane Regions
- Not Available
- Cellular Location
- Mitochondrion matrix
- Gene sequence
>lcl|BSEQ0010122|Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial (HADH) ATGGCCTTCGTCACCAGGCAGTTCATGCGTTCCGTGTCCTCCTCGTCCACCGCCTCGGCC TCGGCCAAGAAGATAATCGTCAAGCACGTGACGGTCATCGGCGGCGGGCTGATGGGCGCC GGCATTGCCCAGGTTGCTGCAGCAACTGGTCACACAGTAGTGTTGGTAGACCAGACAGAG GACATCCTGGCAAAATCCAAAAAGGGAATTGAGGAAAGCCTTAGGAAAGTGGCAAAGAAG AAGTTTGCAGAAAACCCTAAGGCCGGCGATGAATTTGTGGAGAAGACCCTGAGCACCATA GCGACCAGCACGGATGCAGCCTCCGTTGTCCACAGCACAGACTTGGTGGTGGAAGCCATC GTGGAGAATCTGAAGGTGAAAAACGAGCTCTTCAAAAGGCTGGACAAGTTTGCTGCTGAA CATACAATCTTTGCCAGCAACACTTCCTCCTTGCAGATTACAAGCATAGCTAATGCCACC ACCAGACAAGACCGATTCGCTGGCCTCCATTTCTTCAACCCAGTGCCTGTCATGAAACTT GTGGAGGTCATTAAAACACCAATGACCAGCCAGAAGACATTTGAATCTTTGGTAGACTTT AGCAAAGCCCTAGGAAAGCATCCTGTTTCTTGCAAGGACACTCCTGGGTTTATTGTGAAC CGCCTCCTGGTTCCATACCTCATGGAAGCAATCAGGCTGTATGAACGAGACTTCCAAACG TGTGGTGATTCTAACTCGGGTTTGGGCTTTTCTTTAAAAGGTGACGCATCCAAAGAAGAC ATTGACACTGCTATGAAATTAGGAGCCGGTTACCCCATGGGCCCATTTGAGCTTCTAGAT TATGTCGGACTGGATACTACGAAGTTCATCGTGGATGGGTGGCATGAAATGGATGCAGAG AACCCATTACATCAGCCCAGCCCATCCTTAAATAAGCTGGTAGCAGAGAACAAGTTCGGC AAGAAGACTGGAGAAGGATTTTACAAATACAAGTGA
- Chromosome Location
- 4
- Locus
- 4q22-q26
- External Identifiers
Resource Link UniProtKB ID Q16836 UniProtKB Entry Name HCDH_HUMAN GenBank Protein ID 1483511 GenBank Gene ID X96752 GenAtlas ID HADH HGNC ID HGNC:4799 - General References
- Vredendaal PJ, van den Berg IE, Malingre HE, Stroobants AK, Olde Weghuis DE, Berger R: Human short-chain L-3-hydroxyacyl-CoA dehydrogenase: cloning and characterization of the coding sequence. Biochem Biophys Res Commun. 1996 Jun 25;223(3):718-23. [Article]
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- 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]
- Vaca Jacome AS, Rabilloud T, Schaeffer-Reiss C, Rompais M, Ayoub D, Lane L, Bairoch A, Van Dorsselaer A, Carapito C: N-terminome analysis of the human mitochondrial proteome. Proteomics. 2015 Jul;15(14):2519-24. doi: 10.1002/pmic.201400617. Epub 2015 Jun 8. [Article]
- Barycki JJ, O'Brien LK, Bratt JM, Zhang R, Sanishvili R, Strauss AW, Banaszak LJ: Biochemical characterization and crystal structure determination of human heart short chain L-3-hydroxyacyl-CoA dehydrogenase provide insights into catalytic mechanism. Biochemistry. 1999 May 4;38(18):5786-98. [Article]
- Barycki JJ, O'Brien LK, Strauss AW, Banaszak LJ: Sequestration of the active site by interdomain shifting. Crystallographic and spectroscopic evidence for distinct conformations of L-3-hydroxyacyl-CoA dehydrogenase. J Biol Chem. 2000 Sep 1;275(35):27186-96. [Article]
- Yang SY, He XY, Schulz H: 3-Hydroxyacyl-CoA dehydrogenase and short chain 3-hydroxyacyl-CoA dehydrogenase in human health and disease. FEBS J. 2005 Oct;272(19):4874-83. [Article]
- Clayton PT, Eaton S, Aynsley-Green A, Edginton M, Hussain K, Krywawych S, Datta V, Malingre HE, Berger R, van den Berg IE: Hyperinsulinism in short-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency reveals the importance of beta-oxidation in insulin secretion. J Clin Invest. 2001 Aug;108(3):457-65. [Article]
Drug Relations
- Drug Relations
DrugBank ID Name Drug group Pharmacological action? Actions Details DB00157 NADH approved, nutraceutical unknown Details DB03059 Acetoacetyl-CoA experimental unknown Details DB03612 3-Hydroxybutyryl-Coenzyme A experimental unknown Details DB09568 Omega-3-carboxylic acids approved, investigational yes potentiator Details