2-oxoisovalerate dehydrogenase subunit beta, mitochondrial (EC 1.2.4.4) (Branched-chain alpha-keto acid dehydrogenase E1 component beta chain) (BCKDE1B) (BCKDH E1-beta)
1_MAVVA 6_ AAAGW 11_ LLRLR 16_ AAGAE 21_ GHWRR 26_ LPGAG 31_ LARGF 36_ LHPAA 41_ TVEDA 46_ AQRRQ 51_ VAHFT 56_ FQPDP 61_ EPREY 66_ GQTQK 71_ MNLFQ 76_ SVTSA 81_ LDNSL 86_ AKDPT 91_ AVIFG 96_ EDVAF 101_ GGVFR 106_ CTVGL 111_ RDKYG 116_ KDRVF 121_ NTPLC 126_ EQGIV 131_ GFGIG 136_ IAVTG 141_ ATAIA 146_ EIQFA 151_ DYIFP 156_ AFDQI 161_ VNEAA 166_ KYRYR 171_ SGDLF 176_ NCGSL 181_ TIRSP 186_ WGCVG 191_ HGALY 196_ HSQSP 201_ EAFFA 206_ HCPGI 211_ KVVIP 216_ RSPFQ 221_ AKGLL 226_ LSCIE 231_ DKNPC 236_ IFFEP 241_ KILYR 246_ AAAEE 251_ VPIEP 256_ YNIPL 261_ SQAEV 266_ IQEGS 271_ DVTLV 276_ AWGTQ 281_ VHVIR 286_ EVASM 291_ AKEKL 296_ GVSCE 301_ VIDLR 306_ TIIPW 311_ DVDTI 316_ CKSVI 321_ KTGRL 326_ LISHE 331_ APLTG 336_ GFASE 341_ ISSTV 346_ QEECF 351_ LNLEA 356_ PISRV 361_ CGYDT 366_ PFPHI 371_ FEPFY 376_ IPDKW 381_ KCYDA 386_LRKMI
1: Together with BCKDHA forms the heterotetrameric E1 subunit of the mitochondrial branched-chain alpha-ketoacid dehydrogenase (BCKD) complex. The BCKD complex catalyzes the multi-step oxidative decarboxylation of alpha-ketoacids derived from the branched-chain amino-acids valine, leucine and isoleucine producing CO2 and acyl-CoA which is subsequently utilized to produce energy. The E1 subunit catalyzes the first step with the decarboxylation of the alpha-ketoacid forming an enzyme-product intermediate. A reductive acylation mediated by the lipoylamide cofactor of E2 extracts the acyl group from the E1 active site for the next step of the reaction