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NameHydroxyacyl-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 NameHADH
OrganismHuman
Amino acid sequence
>lcl|BSEQ0037004|Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial
MAFVTRQFMRSVSSSSTASASAKKIIVKHVTVIGGGLMGAGIAQVAAATGHTVVLVDQTE
DILAKSKKGIEESLRKVAKKKFAENLKAGDEFVEKTLSTIATSTDAASVVHSTDLVVEAI
VENLKVKNELFKRLDKFAAEHTIFASNTSSLQITSIANATTRQDRFAGLHFFNPVPVMKL
VEVIKTPMTSQKTFESLVDFSKALGKHPVSCKDTPGFIVNRLLVPYLMEAIRLYERGDAS
KEDIDTAMKLGAGYPMGPFELLDYVGLDTTKFIVDGWHEMDAENPLHQPSPSLNKLVAEN
KFGKKTGEGFYKYK
Number of residues314
Molecular Weight34293.275
Theoretical pI9.41
GO Classification
Functions
  • 3-hydroxyacyl-CoA dehydrogenase activity
  • NAD+ binding
Processes
  • cellular lipid metabolic process
  • response to insulin
  • negative regulation of insulin secretion
  • response to drug
  • fatty acid beta-oxidation
  • small molecule metabolic process
  • response to activity
Components
  • cytoplasm
  • mitochondrion
  • mitochondrial inner membrane
  • mitochondrial matrix
  • nucleoplasm
General FunctionNad+ binding
Specific FunctionPlays 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 RegionsNot Available
GenBank Protein ID1483511
UniProtKB IDQ16836
UniProtKB Entry NameHCDH_HUMAN
Cellular LocationMitochondrion 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
GenBank Gene IDX96752
GeneCard IDNot Available
GenAtlas IDHADH
HGNC IDHGNC:4799
Chromosome Location4
Locus4q22-q26
References
  1. 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. 8687463
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  6. 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. 24275569
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