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 |
acyl-CoA metabolic process |
branched-chain amino acid catabolic process |
cellular nitrogen compound metabolic process |
fatty acid metabolic process |
Cellular Component |
mitochondrial matrix |
Function |
adenyl nucleotide binding |
oxidoreductase activity |
fad or fadh2 binding |
oxidoreductase activity, acting on the ch-ch group of donors |
acyl-coa dehydrogenase activity |
binding |
catalytic activity |
nucleoside binding |
purine nucleoside binding |
Molecular Function |
short-branched-chain-acyl-CoA dehydrogenase activity |
electron carrier activity |
acyl-CoA dehydrogenase activity |
flavin adenine dinucleotide binding |
Process |
metabolic process |
oxidation reduction |
|
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 |