Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial (EC 2.3.1.168) (52 kDa mitochondrial autoantigen of primary biliary cirrhosis) (Branched chain 2-oxo-acid dehydrogenase complex component E2) (BCOADC-E2) (Branched-chain alpha-keto acid dehydrogenase complex component E2) (BCKAD-E2) (BCKADE2) (Dihydrolipoamide acetyltransferase component of branched-chain alpha-keto acid dehydrogenase complex) (Dihydrolipoamide branched chain transacylase) (Dihydrolipoyllysine-residue (2-methylpropanoyl)transferase)
1_MAAVR 6_ MLRTW 11_ SRNAG 16_ KLICV 21_ RYFQT 26_ CGNVH 31_ VLKPN 36_ YVCFF 41_ GYPSF 46_ KYSHP 51_ HHFLK 56_ TTAAL 61_ RGQVV 66_ QFKLS 71_ DIGEG 76_ IREVT 81_ VKEWY 86_ VKEGD 91_ TVSQF 96_ DSICE 101_ VQSDK 106_ ASVTI 111_ TSRYD 116_ GVIKK 121_ LYYNL 126_ DDIAY 131_ VGKPL 136_ VDIET 141_ EALKD 146_ SEEDV 151_ VETPA 156_ VSHDE 161_ HTHQE 166_ IKGRK 171_ TLATP 176_ AVRRL 181_ AMENN 186_ IKLSE 191_ VVGSG 196_ KDGRI 201_ LKEDI 206_ LNYLE 211_ KQTGA 216_ ILPPS 221_ PKVEI 226_ MPPPP 231_ KPKDM 236_ TVPIL 241_ VSKPP 246_ VFTGK 251_ DKTEP 256_ IKGFQ 261_ KAMVK 266_ TMSAA 271_ LKIPH 276_ FGYCD 281_ EIDLT 286_ ELVKL 291_ REELK 296_ PIAFA 301_ RGIKL 306_ SFMPF 311_ FLKAA 316_ SLGLL 321_ QFPIL 326_ NASVD 331_ ENCQN 336_ ITYKA 341_ SHNIG 346_ IAMDT 351_ EQGLI 356_ VPNVK 361_ NVQIC 366_ SIFDI 371_ ATELN 376_ RLQKL 381_ GSVGQ 386_ LSTTD 391_ LTGGT 396_ FTLSN 401_ IGSIG 406_ GTFAK 411_ PVIMP 416_ PEVAI 421_ GALGS 426_ IKAIP 431_ RFNQK 436_ GEVYK 441_ AQIMN 446_ VSWSA 451_ DHRVI 456_ DGATM 461_ SRFSN 466_ LWKSY 471_ LENPA 476_FMLLD
1: The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). Within this complex, the catalytic function of this enzyme is to accept, and to transfer to coenzyme A, acyl groups that are generated by the branched-chain alpha-keto acid decarboxylase component