Propionyl-CoA carboxylase alpha chain, mitochondrial (PCCase subunit alpha) (EC 6.4.1.3) (Propanoyl-CoA:carbon dioxide ligase subunit alpha)
1_MAGFW 6_ VGTAP 11_ LVAAG 16_ RRGRW 21_ PPQQL 26_ MLSAA 31_ LRTLK 36_ HVLYY 41_ SRQCL 46_ MVSRN 51_ LGSVG 56_ YDPNE 61_ KTFDK 66_ ILVAN 71_ RGEIA 76_ CRVIR 81_ TCKKM 86_ GIKTV 91_ AIHSD 96_ VDASS 101_ VHVKM 106_ ADEAV 111_ CVGPA 116_ PTSKS 121_ YLNMD 126_ AIMEA 131_ IKKTR 136_ AQAVH 141_ PGYGF 146_ LSENK 151_ EFARC 156_ LAAED 161_ VVFIG 166_ PDTHA 171_ IQAMG 176_ DKIES 181_ KLLAK 186_ KAEVN 191_ TIPGF 196_ DGVVK 201_ DAEEA 206_ VRIAR 211_ EIGYP 216_ VMIKA 221_ SAGGG 226_ GKGMR 231_ IAWDD 236_ EETRD 241_ GFRLS 246_ SQEAA 251_ SSFGD 256_ DRLLI 261_ EKFID 266_ NPRHI 271_ EIQVL 276_ GDKHG 281_ NALWL 286_ NEREC 291_ SIQRR 296_ NQKVV 301_ EEAPS 306_ IFLDA 311_ ETRRA 316_ MGEQA 321_ VALAR 326_ AVKYS 331_ SAGTV 336_ EFLVD 341_ SKKNF 346_ YFLEM 351_ NTRLQ 356_ VEHPV 361_ TECIT 366_ GLDLV 371_ QEMIR 376_ VAKGY 381_ PLRHK 386_ QADIR 391_ INGWA 396_ VECRV 401_ YAEDP 406_ YKSFG 411_ LPSIG 416_ RLSQY 421_ QEPLH 426_ LPGVR 431_ VDSGI 436_ QPGSD 441_ ISIYY 446_ DPMIS 451_ KLITY 456_ GSDRT 461_ EALKR 466_ MADAL 471_ DNYVI 476_ RGVTH 481_ NIALL 486_ REVII 491_ NSRFV 496_ KGDIS 501_ TKFLS 506_ DVYPD 511_ GFKGH 516_ MLTKS 521_ EKNQL 526_ LAIAS 531_ SLFVA 536_ FQLRA 541_ QHFQE 546_ NSRMP 551_ VIKPD 556_ IANWE 561_ LSVKL 566_ HDKVH 571_ TVVAS 576_ NNGSV 581_ FSVEV 586_ DGSKL 591_ NVTST 596_ WNLAS 601_ PLLSV 606_ SVDGT 611_ QRTVQ 616_ CLSRE 621_ AGGNM 626_ SIQFL 631_ GTVYK 636_ VNILT 641_ RLAAE 646_ LNKFM 651_ LEKVT 656_ EDTSS 661_ VLRSP 666_ MPGVV 671_ VAVSV 676_ KPGDA 681_ VAEGQ 686_ EICVI 691_ EAMKM 696_ QNSMT 701_ AGKTG 706_ TVKSV 711_ HCQAG 716_ DTVGE 721_GDLLV
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:6765947, PubMed:8434582). Propionyl-CoA carboxylase catalyzes the carboxylation of propionyl-CoA/propanoyl-CoA to D-methylmalonyl-CoA/(S)-methylmalonyl-CoA (PubMed:10101253, PubMed:6765947, PubMed:8434582). 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)