3-hydroxyisobutyryl-CoA hydrolase, mitochondrial

Name3-hydroxyisobutyryl-CoA hydrolase, mitochondrial
Synonyms
  • 3-hydroxyisobutyryl-coenzyme A hydrolase
  • 3.1.2.4
  • HIB-CoA hydrolase
  • HIBYL-CoA-H
Gene NameHIBCH
OrganismHuman
Amino acid sequence
>lcl|BSEQ0008223|3-hydroxyisobutyryl-CoA hydrolase, mitochondrial
MGQREMWRLMSRFNAFKRTNTILHHLRMSKHTDAAEEVLLEKKGCTGVITLNRPKFLNAL
TLNMIRQIYPQLKKWEQDPETFLIIIKGAGGKAFCAGGDIRVISEAEKAKQKIAPVFFRE
EYMLNNAVGSCQKPYVALIHGITMGGGVGLSVHGQFRVATEKCLFAMPETAIGLFPDVGG
GYFLPRLQGKLGYFLALTGFRLKGRDVYRAGIATHFVDSEKLAMLEEDLLALKSPSKENI
ASVLENYHTESKIDRDKSFILEEHMDKINSCFSANTVEEIIENLQQDGSSFALEQLKVIN
KMSPTSLKITLRQLMEGSSKTLQEVLTMEYRLSQACMRGHDFHEGVRAVLIDKDQSPKWK
PADLKEVTEEDLNNHFKSLGSSDLKF
Number of residues386
Molecular Weight43481.935
Theoretical pI8.36
GO Classification
Functions
  • 3-hydroxyisobutyryl-CoA hydrolase activity
  • hydro-lyase activity
Processes
  • valine catabolic process
  • cellular nitrogen compound metabolic process
  • small molecule metabolic process
  • branched-chain amino acid catabolic process
Components
  • mitochondrial matrix
  • extracellular exosome
General FunctionHydro-lyase activity
Specific FunctionHydrolyzes 3-hydroxyisobutyryl-CoA (HIBYL-CoA), a saline catabolite. Has high activity toward isobutyryl-CoA. Could be an isobutyryl-CoA dehydrogenase that functions in valine catabolism. Also hydrolyzes 3-hydroxypropanoyl-CoA.
Pfam Domain Function
Transmembrane RegionsNot Available
GenBank Protein ID3320120
UniProtKB IDQ6NVY1
UniProtKB Entry NameHIBCH_HUMAN
Cellular LocationMitochondrion
Gene sequence
>lcl|BSEQ0017413|3-hydroxyisobutyryl-CoA hydrolase, mitochondrial (HIBCH)
ATGGGGCAGCGCGAGATGTGGAGGCTCATGTCGAGGTTTAATGCATTCAAAAGGACTAAT
ACCATACTGCACCATTTGAGAATGTCCAAGCACACAGATGCAGCAGAAGAGGTGCTATTG
GAAAAAAAAGGTTGCACGGGAGTCATAACACTAAACAGACCAAAGTTCCTCAATGCACTG
ACTCTTAATATGATTCGGCAGATTTATCCACAGCTAAAGAAGTGGGAACAAGATCCTGAA
ACTTTCCTGATCATTATAAAGGGAGCAGGAGGAAAGGCTTTCTGTGCCGGGGGTGATATC
AGAGTGATCTCGGAAGCTGAAAAGGCAAAACAGAAGATAGCTCCAGTTTTCTTCAGAGAA
GAATATATGCTGAATAATGCTGTTGGTTCTTGCCAGAAACCTTATGTTGCACTTATTCAT
GGAATTACAATGGGTGGGGGAGTTGGTCTCTCAGTCCATGGGCAATTTCGAGTGGCTACA
GAAAAGTGTCTTTTTGCTATGCCAGAAACTGCAATAGGACTGTTCCCTGATGTGGGTGGA
GGTTATTTCTTGCCACGACTCCAAGGAAAACTTGGTTACTTCCTTGCATTAACAGGATTC
AGACTAAAAGGAAGAGATGTGTACAGAGCAGGAATTGCTACACACTTTGTAGATTCTGAA
AAGTTGGCCATGTTAGAGGAAGATTTGTTAGCCTTGAAATCTCCTTCAAAAGAAAATATT
GCATCTGTCTTAGAAAATTACCATACAGAGTCTAAGATTGATCGAGACAAGTCTTTTATA
CTTGAGGAACACATGGACAAAATAAACAGTTGTTTTTCAGCCAATACTGTGGAAGAAATT
ATTGAAAACTTACAGCAAGATGGTTCATCTTTTGCCCTAGAGCAATTGAAGGTAATTAAT
AAAATGTCTCCAACATCTCTAAAGATCACACTAAGGCAACTCATGGAGGGGTCTTCAAAG
ACCTTGCAAGAAGTACTAACTATGGAGTATCGGCTAAGTCAAGCTTGTATGAGAGGTCAT
GACTTTCATGAAGGCGTTAGAGCTGTTTTAATTGATAAAGACCAGAGTCCAAAATGGAAA
CCAGCTGATCTAAAAGAAGTTACTGAGGAAGATTTGAATAATCACTTTAAGTCTTTGGGA
AGCAGTGATTTGAAATTTTGA
GenBank Gene IDU66669
GeneCard IDNot Available
GenAtlas IDNot Available
HGNC IDHGNC:4908
Chromosome Location2
Locus2q32.2
References
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