| Identification |
| HMDB Protein ID
| CDBP01496 |
| Secondary Accession Numbers
| Not Available |
| Name
| Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial |
| Description
| Not Available |
| Synonyms
|
- 2-MEBCAD
- 2-methyl branched chain acyl-CoA dehydrogenase
- 2-methylbutyryl-CoA dehydrogenase
- 2-methylbutyryl-coenzyme A dehydrogenase
- SBCAD
|
| Gene Name
| ACADSB |
| Protein Type
| Enzyme |
| Biological Properties |
| General Function
| Involved in acyl-CoA dehydrogenase activity |
| Specific Function
| Has 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.
|
| GO Classification
|
| Biological Process |
| fatty acid metabolic process |
| acyl-CoA metabolic process |
| branched-chain amino acid catabolic process |
| cellular nitrogen compound metabolic process |
| Cellular Component |
| mitochondrial matrix |
| Function |
| oxidoreductase activity, acting on the ch-ch group of donors |
| acyl-coa dehydrogenase activity |
| binding |
| catalytic activity |
| nucleoside binding |
| purine nucleoside binding |
| adenyl nucleotide binding |
| oxidoreductase activity |
| fad or fadh2 binding |
| Molecular Function |
| short-branched-chain-acyl-CoA dehydrogenase activity |
| electron carrier activity |
| acyl-CoA dehydrogenase activity |
| flavin adenine dinucleotide binding |
| Process |
| oxidation reduction |
| metabolic process |
|
| Cellular Location
|
- Mitochondrion matrix
|
| Pathways
|
| Name | SMPDB/Pathwhiz | KEGG | | mitochondrial fatty acid beta-oxidation | Not Available | Not Available | | Fatty acid Metabolism |    |  | | Ethylmalonic Encephalopathy |    | Not Available | | Glutaric Aciduria Type I |    | Not Available | | Short Chain Acyl CoA Dehydrogenase Deficiency (SCAD Deficiency) |    | Not Available |
|
| Gene Properties |
| Chromosome Location
| 10 |
| Locus
| 10q26.13 |
| SNPs
| ACADSB |
| Gene Sequence
|
>1299 bp
ATGGAGGGCCTGGCAGTGCGGTTGCTGCGCGGCAGCAGGCTGCTAAGAAGAAATTTCCTG
ACTTGTTTGTCTTCTTGGAAGATTCCTCCTCATGTCTCAAAATCTTCCCAGTCAGAAGCT
CTACTCAATATAACAAATAATGGAATACACTTTGCTCCCCTGCAAACATTTACAGATGAG
GAAATGATGATAAAGAGTTCAGTTAAAAAATTTGCTCAGGAACAAATTGCACCTTTGGTT
TCAACCATGGATGAAAATTCGAAAATGGAGAAATCAGTAATACAAGGATTATTTCAACAA
GGGTTGATGGGTATTGAAGTTGACCCAGAATATGGAGGCACAGGAGCTTCTTTTTTATCC
ACTGTGCTCGTGATAGAGGAATTAGCCAAAGTTGATGCATCTGTGGCTGTCTTTTGTGAG
ATCCAGAACACATTAATTAACACACTGATTAGAAAACATGGAACAGAAGAACAAAAGGCC
ACCTATTTGCCTCAGCTCACTACAGAAAAAGTAGGAAGTTTCTGCCTTTCAGAGGCTGGA
GCAGGTAGTGACTCATTTGCTTTGAAGACCAGAGCTGATAAAGAGGGAGATTATTATGTC
CTCAATGGATCAAAGATGTGGATCAGCAGTGCTGAGCATGCAGGGCTCTTTCTGGTGATG
GCAAATGTAGACCCTACCATTGGATATAAGGGAATTACCTCCTTCTTAGTAGATCGTGAT
ACTCCGGGCCTTCATATAGGGAAACCTGAAAACAAATTGGGGCTCAGAGCTTCTTCCACC
TGCCCGTTAACATTCGAAAATGTCAAGGTTCCAGAAGCCAATATCTTGGGACAAATTGGA
CATGGCTATAAGTATGCCATAGGGAGTCTCAATGAAGGTAGAATAGGAATTGCTGCACAG
ATGCTGGGACTGGCGCAAGGATGTTTTGACTACACTATTCCATATATTAAAGAAAGGATA
CAATTTGGCAAAAGACTATTTGATTTTCAGGGCCTCCAACACCAAGTGGCTCACGTGGCC
ACCCAGCTGGAAGCTGCAAGATTACTAACATACAATGCTGCTAGGCTTTTAGAAGCTGGA
AAGCCATTCATAAAAGAAGCGTCAATGGCCAAATACTATGCATCAGAGATTGCAGGACAA
ACAACGAGTAAATGTATCGAGTGGATGGGGGGAGTAGGCTACACCAAAGATTACCCTGTG
GAGAAATACTTCCGAGATGCAAAGATTGGTACGATATATGAAGGAGCTTCCAACATCCAG
TTGAACACCATTGCAAAGCATATCGATGCAGAATACTGA
|
| Protein Properties |
| Number of Residues
| 432 |
| Molecular Weight
| 47485.035 |
| Theoretical pI
| 6.991 |
| Pfam Domain Function
|
|
| Signals
|
Not Available
|
|
Transmembrane Regions
|
Not Available
|
| Protein Sequence
|
>Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
MEGLAVRLLRGSRLLRRNFLTCLSSWKIPPHVSKSSQSEALLNITNNGIHFAPLQTFTDE
EMMIKSSVKKFAQEQIAPLVSTMDENSKMEKSVIQGLFQQGLMGIEVDPEYGGTGASFLS
TVLVIEELAKVDASVAVFCEIQNTLINTLIRKHGTEEQKATYLPQLTTEKVGSFCLSEAG
AGSDSFALKTRADKEGDYYVLNGSKMWISSAEHAGLFLVMANVDPTIGYKGITSFLVDRD
TPGLHIGKPENKLGLRASSTCPLTFENVKVPEANILGQIGHGYKYAIGSLNEGRIGIAAQ
MLGLAQGCFDYTIPYIKERIQFGKRLFDFQGLQHQVAHVATQLEAARLLTYNAARLLEAG
KPFIKEASMAKYYASEIAGQTTSKCIEWMGGVGYTKDYPVEKYFRDAKIGTIYEGASNIQ
LNTIAKHIDAEY
|
| External Links |
| GenBank ID Protein
| Not Available |
| UniProtKB/Swiss-Prot ID
| P45954 |
| UniProtKB/Swiss-Prot Entry Name
| ACDSB_HUMAN |
| PDB IDs
|
|
| GenBank Gene ID
| U12778 |
| GeneCard ID
| ACADSB |
| GenAtlas ID
| ACADSB |
| HGNC ID
| HGNC:91 |
| References |
| General References
| Not Available |