UniProt Accession

 ACAD8_HUMAN; Q9UKU7; Q9BUS8 

Genbank Protein ID

 AF126245; AF260689; AF260679; AF260680; AF260681; AF260682; AF260683; AF260684; AF260685; AF260686; AF260687; AF260688; AK000359; BC001964 

Genbank Nucleotide ID

 AAF12736.1; AAF97922.1; AAF97922.1; AAF97922.1; AAF97922.1; AAF97922.1; AAF97922.1; AAF97922.1; AAF97922.1; AAF97922.1; AAF97922.1; AAF97922.1; BAA91109.1; AAH01964.1 

Protein Name

 Isobutyryl-CoA dehydrogenase, mitochondrial 

Protein Synonyms/Alias

 Activator-recruited cofactor 42 kDa component; ARC42; Acyl-CoA dehydrogenase family member 8; ACAD-8 

Gene Name

 ACAD8 

Gene Synonyms/Alias

 ARC42; IBD 

Created Date

 July 27, 2013 

Organism

 Homo sapiens (Human) 

NCBI Taxa ID

 9606 

Lysine Modification

Position	Peptide	Type	References
144	GNEEQRHKFCPPLCT	ubiquitination	[1]
177	ASLLTSAKKQGDHYI	ubiquitination	[1]
178	SLLTSAKKQGDHYIL	ubiquitination	[1]
213	RTGGPGPKGISCIVV	ubiquitination	[1]
232	PGLSFGKKEKKVGWN	ubiquitination	[1]
235	SFGKKEKKVGWNSQP	ubiquitination	[1]
303	RDHLNVRKQFGEPLA	ubiquitination	[1]
345	VALQEERKDAVALCS	ubiquitination	[1]

Reference

 [1] Refined preparation and use of anti-diglycine remnant (K-ε-GG) antibody enables routine quantification of 10,000s of ubiquitination sites in single proteomics experiments.
 Udeshi ND, Svinkina T, Mertins P, Kuhn E, Mani DR, Qiao JW, Carr SA.
 Mol Cell Proteomics. 2013 Mar;12(3):825-31. [PMID: 23266961] 

Functional Description

 Has very high activity toward isobutyryl-CoA. Is an isobutyryl-CoA dehydrogenase that functions in valine catabolism. Plays a role in transcriptional coactivation within the ARC complex. 

Sequence Annotation

 NP_BIND 158 167 FAD.
 NP_BIND 191 193 FAD.
 NP_BIND 312 313 FAD; shared with dimeric partner.
 NP_BIND 371 375 FAD; shared with dimeric partner.
 NP_BIND 400 402 FAD.
 REGION 274 277 Substrate binding.
 ACT_SITE 398 398 Proton acceptor.
 BINDING 167 167 Substrate; via carbonyl oxygen.
 BINDING 302 302 FAD; shared with dimeric partner.
 BINDING 399 399 Substrate; via amide nitrogen.
 BINDING 410 410 Substrate.  

Keyword

 3D-structure; Activator; Branched-chain amino acid catabolism; Complete proteome; Direct protein sequencing; Disease mutation; FAD; Flavoprotein; Mitochondrion; Oxidoreductase; Reference proteome; Transcription; Transcription regulation; Transit peptide. 

Sequence Source

 UniProt (SWISSPROT/TrEMBL); GenBank; EMBL 

Protein Length

 415 AA 

Protein Sequence

Gene Ontology

 GO:0005759; C:mitochondrial matrix; TAS:Reactome.
 GO:0003995; F:acyl-CoA dehydrogenase activity; EXP:Reactome.
 GO:0050660; F:flavin adenine dinucleotide binding; IEA:InterPro.
 GO:0009083; P:branched-chain amino acid catabolic process; TAS:Reactome.
 GO:0034641; P:cellular nitrogen compound metabolic process; TAS:Reactome.
 GO:0006629; P:lipid metabolic process; TAS:ProtInc.
 GO:0006355; P:regulation of transcription, DNA-dependent; IEA:UniProtKB-KW.
 GO:0006351; P:transcription, DNA-dependent; IEA:UniProtKB-KW.
 GO:0006574; P:valine catabolic process; IEA:UniProtKB-UniPathway. 

Interpro

 IPR006089; Acyl-CoA_DH_CS.
 IPR006092; Acyl-CoA_DH_N.
 IPR006090; Acyl-CoA_Oxase/DH_1.
 IPR006091; Acyl-CoA_Oxase/DH_cen-dom.
 IPR009075; AcylCo_DH/oxidase_C.
 IPR013786; AcylCoA_DH/ox_N.
 IPR009100; AcylCoA_DH/oxidase. 

Pfam

 PF00441; Acyl-CoA_dh_1
 PF02770; Acyl-CoA_dh_M
 PF02771; Acyl-CoA_dh_N 

SMART

  

PROSITE

 PS00072; ACYL_COA_DH_1
 PS00073; ACYL_COA_DH_2 

PRINTS