Propionyl-CoA carboxylase beta chain, mitochondrial (PCCase subunit beta) (EC 6.4.1.3) (Propanoyl-CoA:carbon dioxide ligase subunit beta)
1_MAAAL 6_ RVAAV 11_ GARLS 16_ VLASG 21_ LRAAV 26_ RSLCS 31_ QATSV 36_ NERIE 41_ NKRRT 46_ ALLGG 51_ GQRRI 56_ DAQHK 61_ RGKLT 66_ ARERI 71_ SLLLD 76_ PGSFV 81_ ESDMF 86_ VEHRC 91_ ADFGM 96_ AADKN 101_ KFPGD 106_ SVVTG 111_ RGRIN 116_ GRLVY 121_ VFSQD 126_ FTVFG 131_ GSLSG 136_ AHAQK 141_ ICKIM 146_ DQAIT 151_ VGAPV 156_ IGLND 161_ SGGAR 166_ IQEGV 171_ ESLAG 176_ YADIF 181_ LRNVT 186_ ASGVI 191_ PQISL 196_ IMGPC 201_ AGGAV 206_ YSPAL 211_ TDFTF 216_ MVKDT 221_ SYLFI 226_ TGPDV 231_ VKSVT 236_ NEDVT 241_ QEELG 246_ GAKTH 251_ TTMSG 256_ VAHRA 261_ FENDV 266_ DALCN 271_ LRDFF 276_ NYLPL 281_ SSQDP 286_ APVRE 291_ CHDPS 296_ DRLVP 301_ ELDTI 306_ VPLES 311_ TKAYN 316_ MVDII 321_ HSVVD 326_ EREFF 331_ EIMPN 336_ YAKNI 341_ IVGFA 346_ RMNGR 351_ TVGIV 356_ GNQPK 361_ VASGC 366_ LDINS 371_ SVKGA 376_ RFVRF 381_ CDAFN 386_ IPLIT 391_ FVDVP 396_ GFLPG 401_ TAQEY 406_ GGIIR 411_ HGAKL 416_ LYAFA 421_ EATVP 426_ KVTVI 431_ TRKAY 436_ GGAYD 441_ VMSSK 446_ HLCGD 451_ TNYAW 456_ PTAEI 461_ AVMGA 466_ KGAVE 471_ IIFKG 476_ HENVE 481_ AAQAE 486_ YIEKF 491_ ANPFP 496_ AAVRG 501_ FVDDI 506_ IQPSS 511_ TRARI 516_ CCDLD 521_ VLASK 526_ KVQRP 531_WRKHA
1: This is one of the 2 subunits of the biotin-dependent propionyl-CoA carboxylase (PCC), a mitochondrial enzyme involved in the catabolism of odd chain fatty acids, branched-chain amino acids isoleucine, threonine, methionine, and valine and other metabolites (PubMed:15890657, PubMed:6765947). Propionyl-CoA carboxylase catalyzes the carboxylation of propionyl-CoA/propanoyl-CoA to D-methylmalonyl-CoA/(S)-methylmalonyl-CoA (PubMed:15890657, PubMed:6765947). Within the holoenzyme, the alpha subunit catalyzes the ATP-dependent carboxylation of the biotin carried by the biotin carboxyl carrier (BCC) domain, while the beta subunit then transfers the carboxyl group from carboxylated biotin to propionyl-CoA (By similarity). Propionyl-CoA carboxylase also significantly acts on butyryl-CoA/butanoyl-CoA, which is converted to ethylmalonyl-CoA/(2S)-ethylmalonyl-CoA at a much lower rate (PubMed:6765947). Other alternative minor substrates include (2E)-butenoyl-CoA/crotonoyl-CoA (By similarity)