Gene/Proteome Database (LMPD)

LMPD ID
LMP001132
Gene ID
36
Species
Homo sapiens (Human)
Gene Name
acyl-CoA dehydrogenase, short/branched chain
Gene Symbol
ACADSB
Synonyms
2-MEBCAD; ACAD7; SBCAD
Alternate Names
short/branched chain specific acyl-CoA dehydrogenase, mitochondrial; 2-methylbutyryl-coenzyme A dehydrogenase; 2-methyl branched chain acyl-CoA dehydrogenase; acyl-Coenzyme A dehydrogenase, short/branched chain
Chromosome
10
Map Location
10q26.13
EC Number
1.3.8.5
Summary
Short/branched chain acyl-CoA dehydrogenase(ACADSB) is a member of the acyl-CoA dehydrogenase family of enzymes that catalyze the dehydrogenation of acyl-CoA derivatives in the metabolism of fatty acids or branch chained amino acids. Substrate specificity is the primary characteristic used to define members of this gene family. The ACADSB gene product has the greatest activity towards the short branched chain acyl-CoA derivative, (S)-2-methylbutyryl-CoA, but also reacts significantly with other 2-methyl branched chain substrates and with short straight chain acyl-CoAs. The cDNA encodes for a mitochondrial precursor protein which is cleaved upon mitochondrial import and predicted to yield a mature peptide of approximately 43.7-KDa. [provided by RefSeq, Jul 2008]
Orthologs
View orthologs and multiple alignments for ACADSB

Proteins

short/branched chain specific acyl-CoA dehydrogenase, mitochondrial precursor
Refseq ID NP_001600
Protein GI 4501859
UniProt ID P45954
mRNA ID NM_001609
Length 432
RefSeq Status REVIEWED
MEGLAVRLLRGSRLLRRNFLTCLSSWKIPPHVSKSSQSEALLNITNNGIHFAPLQTFTDEEMMIKSSVKKFAQEQIAPLVSTMDENSKMEKSVIQGLFQQGLMGIEVDPEYGGTGASFLSTVLVIEELAKVDASVAVFCEIQNTLINTLIRKHGTEEQKATYLPQLTTEKVGSFCLSEAGAGSDSFALKTRADKEGDYYVLNGSKMWISSAEHAGLFLVMANVDPTIGYKGITSFLVDRDTPGLHIGKPENKLGLRASSTCPLTFENVKVPEANILGQIGHGYKYAIGSLNEGRIGIAAQMLGLAQGCFDYTIPYIKERIQFGKRLFDFQGLQHQVAHVATQLEAARLLTYNAARLLEAGKPFIKEASMAKYYASEIAGQTTSKCIEWMGGVGYTKDYPVEKYFRDAKIGTIYEGASNIQLNTIAKHIDAEY
transit_peptide: 1..33 calculated_mol_wt: 3808 peptide sequence: MEGLAVRLLRGSRLLRRNFLTCLSSWKIPPHVS mat_peptide: 34..432 product: short/branched chain specific acyl-CoA dehydrogenase, mitochondrial calculated_mol_wt: 43696 peptide sequence: KSSQSEALLNITNNGIHFAPLQTFTDEEMMIKSSVKKFAQEQIAPLVSTMDENSKMEKSVIQGLFQQGLMGIEVDPEYGGTGASFLSTVLVIEELAKVDASVAVFCEIQNTLINTLIRKHGTEEQKATYLPQLTTEKVGSFCLSEAGAGSDSFALKTRADKEGDYYVLNGSKMWISSAEHAGLFLVMANVDPTIGYKGITSFLVDRDTPGLHIGKPENKLGLRASSTCPLTFENVKVPEANILGQIGHGYKYAIGSLNEGRIGIAAQMLGLAQGCFDYTIPYIKERIQFGKRLFDFQGLQHQVAHVATQLEAARLLTYNAARLLEAGKPFIKEASMAKYYASEIAGQTTSKCIEWMGGVGYTKDYPVEKYFRDAKIGTIYEGASNIQLNTIAKHIDAEY

Gene Information

Entrez Gene ID
36
Gene Name
acyl-CoA dehydrogenase, short/branched chain
Gene Symbol
ACADSB
Species
Homo sapiens

Gene Ontology (GO Annotations)

GO ID Source Type Description
GO:0070062 IDA:UniProt C extracellular vesicular exosome
GO:0005759 TAS:Reactome C mitochondrial matrix
GO:0005739 IDA:HPA C mitochondrion
GO:0003995 EXP:Reactome F acyl-CoA dehydrogenase activity
GO:0050660 IEA:InterPro F flavin adenine dinucleotide binding
GO:0009083 TAS:Reactome P branched-chain amino acid catabolic process
GO:0034641 TAS:Reactome P cellular nitrogen compound metabolic process
GO:0006631 TAS:ProtInc P fatty acid metabolic process
GO:0044281 TAS:Reactome P small molecule metabolic process

KEGG Pathway Links

KEGG Pathway ID Description
hsa00071 Fatty acid degradation
hsa01212 Fatty acid metabolism

BIOCYC Pathway Links

BIOCYC Pathway ID Description
ILEUDEG-PWY isoleucine degradation I

REACTOME Pathway Links

REACTOME Pathway ID Description
REACT_197 Branched-chain amino acid catabolism

Domain Information

InterPro Annotations

Accession Description
IPR006089 Acyl-CoA dehydrogenase, conserved site
IPR009075 Acyl-CoA dehydrogenase/oxidase C-terminal
IPR013786 Acyl-CoA dehydrogenase/oxidase, N-terminal
IPR009100 Acyl-CoA dehydrogenase/oxidase, N-terminal and middle domain
IPR006091 Acyl-CoA oxidase/dehydrogenase, central domain

UniProt Annotations

Entry Information

Gene Name
acyl-CoA dehydrogenase, short/branched chain
Protein Entry
ACDSB_HUMAN
UniProt ID
P45954
Species
Human

Comments

Comment Type Description
Alternative Products Event=Alternative splicing; Named isoforms=2; Name=1; IsoId=P45954-1; Sequence=Displayed; Name=2; IsoId=P45954-2; Sequence=VSP_055778; Note=No experimental confirmation available.;
Catalytic Activity 2-methylbutanoyl-CoA + electron-transfer flavoprotein = (E)-2-methylbut-2-enoyl-CoA + reduced electron- transfer flavoprotein + H(+).
Catalytic Activity Acyl-CoA + acceptor = 2,3-dehydroacyl-CoA + reduced acceptor.
Cofactor Name=FAD; Xref=ChEBI
Disease Short/branched-chain acyl-CoA dehydrogenase deficiency (SBCADD) [MIM
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.
Pathway Lipid metabolism; mitochondrial fatty acid beta- oxidation.
Similarity Belongs to the acyl-CoA dehydrogenase family.
Subcellular Location Mitochondrion matrix.
Subunit Homotetramer.
Tissue Specificity Ubiquitous.

Identical and Related Proteins

Unique RefSeq proteins for LMP001132 (as displayed in Record Overview)

Protein GI Database Accession Length Protein Name
4501859 RefSeq NP_001600 432 short/branched chain specific acyl-CoA dehydrogenase, mitochondrial precursor

Identical Sequences to LMP001132 proteins

Reference Database Accession Length Protein Name
GI:4501859 GenBank AEQ84556.1 432 Sequence 49 from patent US 8039587
GI:4501859 GenBank AEU53419.1 432 Sequence 49 from patent US 8062891
GI:4501859 GenBank AGO72372.1 432 Sequence 49 from patent US 8470972
GI:4501859 GenBank AGU32363.1 432 Sequence 49 from patent US 8507277
GI:4501859 GenBank AGX48267.1 432 Sequence 49 from patent US 8541550
GI:4501859 GenBank AHE01090.1 432 Sequence 56006 from patent US 8586006

Related Sequences to LMP001132 proteins

Reference Database Accession Length Protein Name
GI:4501859 GenBank AAH13756.1 432 Acyl-Coenzyme A dehydrogenase, short/branched chain [Homo sapiens]
GI:4501859 GenBank ADQ31765.1 432 acyl-Coenzyme A dehydrogenase, short/branched chain, partial [synthetic construct]
GI:4501859 GenBank JAA11326.1 432 acyl-CoA dehydrogenase, short/branched chain [Pan troglodytes]
GI:4501859 GenBank JAA13375.1 432 acyl-CoA dehydrogenase, short/branched chain [Pan troglodytes]
GI:4501859 GenBank AIC53942.1 432 ACADSB, partial [synthetic construct]
GI:4501859 RefSeq XP_009457693.1 432 PREDICTED: short/branched chain specific acyl-CoA dehydrogenase, mitochondrial [Pan troglodytes]