alpha-methylbutyryl-CoA + FAD => tiglyl-CoA + FADH2

Stable Identifier
R-HSA-70800
Type
Reaction [transition]
Species
Homo sapiens
Compartment
Locations in the PathwayBrowser
General
SVG |   | PPTX  | SBGN
Click the image above or here to open this reaction in the Pathway Browser
The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout

Mitochondrial 2-methyl branched chain acyl-CoA dehydrogenase (ACADSB) catalyzes the reaction of alpha-methylbutyryl-CoA and FAD to form 'tiglyl-CoA and FADH2 (Andresen et al. 2000; Gibson et al. 2000). Unpublished crystallographic data (PDB 2JIF) indicate that the enzyme is a tetramer of ACADSB polypeptides whose aminoterminal 51 residues, a mitochondrial targeting sequence, have been removed.

Literature References
PubMed ID Title Journal Year
11013134 Isolated 2-methylbutyrylglycinuria caused by short/branched-chain acyl-CoA dehydrogenase deficiency: identification of a new enzyme defect, resolution of its molecular basis, and evidence for distinct acyl-CoA dehydrogenases in isoleucine and valine metabolism.

Andresen, BS, Christensen, E, Corydon, TJ, Bross, P, Pilgaard, B, Ruiter, JP, Simonsen, H, Winter, V, Knudsen, I, Schroeder, LD, Gregersen, N, Skovby, F

Am J Hum Genet 2000
10832746 2-Methylbutyryl-coenzyme A dehydrogenase deficiency: a new inborn error of L-isoleucine metabolism.

Gibson, KM, Burlingame, TG, Hogema, B, Jakobs, C, Schutgens, RB, Millington, D, Roe, DS, Sweetman, L, Steiner, RD, Linck, L, Pohowalla, P, Sacks, M, Kiss, D, Rinaldo, P, Vockley, J

Pediatr Res 2000
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
acyl-CoA dehydrogenase activity of ACADSB tetramer [mitochondrial matrix]
Physical Entity
Activity
Orthologous Events