2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial (EC 1.2.4.4) (Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain) (BCKDE1A) (BCKDH E1-alpha)
1_MAVAI 6_ AAARV 11_ WRLNR 16_ GLSQA 21_ ALLLL 26_ RQPGA 31_ RGLAR 36_ SHPPR 41_ QQQQF 46_ SSLDD 51_ KPQFP 56_ GASAE 61_ FIDKL 66_ EFIQP 71_ NVISG 76_ IPIYR 81_ VMDRQ 86_ GQIIN 91_ PSEDP 96_ HLPKE 101_ KVLKL 106_ YKSMT 111_ LLNTM 116_ DRILY 121_ ESQRQ 126_ GRISF 131_ YMTNY 136_ GEEGT 141_ HVGSA 146_ AALDN 151_ TDLVF 156_ GQYRE 161_ AGVLM 166_ YRDYP 171_ LELFM 176_ AQCYG 181_ NISDL 186_ GKGRQ 191_ MPVHY 196_ GCKER 201_ HFVTI 206_ SSPLA 211_ TQIPQ 216_ AVGAA 221_ YAAKR 226_ ANANR 231_ VVICY 236_ FGEGA 241_ ASEGD 246_ AHAGF 251_ NFAAT 256_ LECPI 261_ IFFCR 266_ NNGYA 271_ ISTPT 276_ SEQYR 281_ GDGIA 286_ ARGPG 291_ YGIMS 296_ IRVDG 301_ NDVFA 306_ VYNAT 311_ KEARR 316_ RAVAE 321_ NQPFL 326_ IEAMT 331_ YRIGH 336_ HSTSD 341_ DSSAY 346_ RSVDE 351_ VNYWD 356_ KQDHP 361_ ISRLR 366_ HYLLS 371_ QGWWD 376_ EEQEK 381_ AWRKQ 386_ SRRKV 391_ MEAFE 396_ QAERK 401_ PKPNP 406_ NLLFS 411_ DVYQE 416_ MPAQL 421_ RKQQE 426_ SLARH 431_ LQTYG 436_EHYPL
1: Together with BCKDHB 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