Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial

NameShort/branched chain specific acyl-CoA dehydrogenase, mitochondrial
Synonyms
  • 1.3.8.5
  • 2-MEBCAD
  • 2-methyl branched chain acyl-CoA dehydrogenase
  • 2-methylbutyryl-CoA dehydrogenase
  • 2-methylbutyryl-coenzyme A dehydrogenase
  • SBCAD
Gene NameACADSB
OrganismHuman
Amino acid sequence
>lcl|BSEQ0001144|Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
MEGLAVRLLRGSRLLRRNFLTCLSSWKIPPHVSKSSQSEALLNITNNGIHFAPLQTFTDE
EMMIKSSVKKFAQEQIAPLVSTMDENSKMEKSVIQGLFQQGLMGIEVDPEYGGTGASFLS
TVLVIEELAKVDASVAVFCEIQNTLINTLIRKHGTEEQKATYLPQLTTEKVGSFCLSEAG
AGSDSFALKTRADKEGDYYVLNGSKMWISSAEHAGLFLVMANVDPTIGYKGITSFLVDRD
TPGLHIGKPENKLGLRASSTCPLTFENVKVPEANILGQIGHGYKYAIGSLNEGRIGIAAQ
MLGLAQGCFDYTIPYIKERIQFGKRLFDFQGLQHQVAHVATQLEAARLLTYNAARLLEAG
KPFIKEASMAKYYASEIAGQTTSKCIEWMGGVGYTKDYPVEKYFRDAKIGTIYEGASNIQ
LNTIAKHIDAEY
Number of residues432
Molecular Weight47485.035
Theoretical pI7.0
GO Classification
Functions
  • electron carrier activity
  • flavin adenine dinucleotide binding
  • fatty-acyl-CoA binding
  • oxidoreductase activity, acting on the CH-CH group of donors, with a flavin as acceptor
  • acyl-CoA dehydrogenase activity
Processes
  • cellular nitrogen compound metabolic process
  • small molecule metabolic process
  • branched-chain amino acid catabolic process
  • fatty acid metabolic process
  • fatty acid beta-oxidation using acyl-CoA dehydrogenase
  • lipid homeostasis
Components
  • extracellular exosome
  • mitochondrion
  • mitochondrial matrix
General FunctionOxidoreductase activity, acting on the ch-ch group of donors, with a flavin as acceptor
Specific FunctionHas greatest activity toward short branched chain acyl-CoA derivative such as (s)-2-methylbutyryl-CoA, isobutyryl-CoA, and 2-methylhexanoyl-CoA as well as toward short straight chain acyl-CoAs such as butyryl-CoA and hexanoyl-CoA. Can use valproyl-CoA as substrate and may play a role in controlling the metabolic flux of valproic acid in the development of toxicity of this agent.
Pfam Domain Function
Transmembrane RegionsNot Available
GenBank Protein ID531391
UniProtKB IDP45954
UniProtKB Entry NameACDSB_HUMAN
Cellular LocationMitochondrion matrix
Gene sequence
>lcl|BSEQ0018973|Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial (ACADSB)
ATGGAGGGCCTGGCAGTGCGGTTGCTGCGCGGCAGCAGGCTGCTAAGAAGAAATTTCCTG
ACTTGTTTGTCTTCTTGGAAGATTCCTCCTCATGTCTCAAAATCTTCCCAGTCAGAAGCT
CTACTCAATATAACAAATAATGGAATACACTTTGCTCCCCTGCAAACATTTACAGATGAG
GAAATGATGATAAAGAGTTCAGTTAAAAAATTTGCTCAGGAACAAATTGCACCTTTGGTT
TCAACCATGGATGAAAATTCGAAAATGGAGAAATCAGTAATACAAGGATTATTTCAACAA
GGGTTGATGGGTATTGAAGTTGACCCAGAATATGGAGGCACAGGAGCTTCATTTTTATCC
ACTGTGCTCGTGATAGAGGAATTAGCCAAAGTTGATGCATCTGTGGCTGTCTTTTGTGAG
ATCCAGAACACATTAATTAACACACTGATTAGAAAACATGGAACAGAAGAACAAAAGGCC
ACCTATTTGCCTCAGCTCACTACAGAAAAAGTAGGAAGTTTCTGCCTTTCAGAGGCTGGA
GCAGGTAGTGACTCATTTGCTTTGAAGACCAGAGCTGATAAAGAGGGAGATTATTATGTC
CTCAATGGATCAAAGATGTGGATCAGCAGTGCTGAGCACGCAGGGCTCTTTCTGGTGATG
GCAAATGTAGACCCTACCATTGGATATAAGGGAATTACCTCCTTCTTAGTAGATCGTGAT
ACTCCGGGCCTTCATATAGGGAAACCTGAAAACAAATTGGGGCTCAGAGCTTCTTCCACC
TGCCCGTTAACATTCGAAAATGTCAAGGTTCCAGAAGCCAATATCTTGGGACAAATTGGA
CATGGCTATAAGTATGCCATAGGGAGTCTCAATGAAGGTAGAATAGGAATTGCTGCACAG
ATGCTGGGACTGGCGCAAGGATGTTTTGACTACACTATTCCATATATTAAAGAAAGGATA
CAATTTGGCAAAAGACTATTTGATTTTCAGGGCCTCCAACACCAAGTGGCTCACGTGGCC
ACCCAGCTGGAAGCTGCAAGATTACTAACATACAATGCTGCTAGGCTTTTAGAAGCTGGA
AAGCCATTCATAAAAGAAGCGTCAATGGCCAAATACTATGCATCAGAGATTGCAGGACAA
ACAACGAGTAAATGTATCGAGTGGATGGGGGGAGTAGGCTACACCAAAGATTACCCTGTG
GAGAAATACTTCCGAGATGCAAAGATTGGTACGATATATGAAGGAGCTTCCAACATCCAG
TTGAACACCATTGCAAAGCATATCGATGCAGAATACTGA
GenBank Gene IDU12778
GeneCard IDNot Available
GenAtlas IDACADSB
HGNC IDHGNC:91
Chromosome Location10
Locus10q26.13
References
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