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Overview

Uniprot IDA0A0G2K401
Protein NamePropionyl-CoA carboxylase alpha chain, mitochondrial
Gene NamePcca
OrganismRattus norvegicus

Kla Sites from experimental identification

Position Flanking peptide
183 KLLAKRAKVNTIPGF

Function

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. Propionyl-CoA carboxylase catalyzes the carboxylation of propionyl-CoA/propanoyl-CoA to D-methylmalonyl-CoA/(S)-methylmalonyl-CoA. 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. Propionyl-CoA carboxylase also significantly acts on butyryl-CoA/butanoyl-CoA, which is converted to ethylmalonyl-CoA/(2S)-ethylmalonyl-CoA. Other alternative minor substrates include (2E)-butenoyl-CoA/crotonoyl-CoA

Protein Sequence

10 MAGLWVRTVA 20 LLAARRHWRR 30 SSQQLLWTLK 40 RAPVYSQQCL 50 VVSRSLSSVE 60 YEPKEKTFDK 70 ILIANRGEIA 80 CRVIKTCRKM 90 GIRTVAIHSD 100 VDASSVHVKM 110 ADEAVCVGPA 120 PTSKSYLNMD 130 AIMEAIKKTG 140 AQAVHPGYGF 150 LSENKEFAKC 160 LAAEDVTFIG 170 PDTHAIQAMG 180 DKIESKLLAK 190 RAKVNTIPGF 200 DGVVKDADEA 210 VRIAREIGYP 220 VMIKASAGGG 230 GKGMRIAWDD 240 EETRDGFRFS 250 SQEAASSFGD 260 DRLLIEKFID 270 NPRHIEIQVL 280 GDKHGNALWL 290 NERECSIQRR 300 NQKVVEEAPS 310 IFLDPETRRA 320 MGEQAVALAK 330 AVKYSSAGTV 340 EFLVDSQKNF 350 YFLEMNTRLQ 360 VEHPVTECIT 370 GLDLVQEMIL 380 VAKGYPLRHK 390 QEDIPISGWA 400 VECRVYAEDP 410 YKSFGLPSIG 420 RLSQYQEPIH 430 LPGLVTYGSD 440 RAEALKRMED 450 ALDSYVIRGV 460 THNIPLLREV 470 IINTRFVKGD 480 ISTKFLSDVY 490 PDGFKGHMLT 500 PSERDQLLAI 510 ASSLFVASQL 520 RAQRFQEHSR 530 VPVIRPDVAK 540 WELSVKLHDE 550 DHTVVASNNG 560 PTFNVEVDGS 570 KLNVTSTWNL 580 ASPLLSVNVD 590 GTQRTVQCLS 600 RDAGGNMSIQ 610 FLGTVYKVHI 620 LTKLAAELNK 630 FMLEKVPKDT 640 SSTLCSPMPG 650 VVVAVSVKPG 660 DMVAEGQEIC 670 VIEAMKMQNS 680 MTAGKMGKVK 690 LVHCKAGDTV 700 GEGDLLVELE

Gene Ontology

Classification GO ID Description
Cellular Component GO:0005759 mitochondrial matrix
Molecular Function GO:0005524 ATP binding
Molecular Function GO:0046872 metal ion binding
Molecular Function GO:0004658 propionyl-CoA carboxylase activity
Biological Process GO:0019752 carboxylic acid metabolic process
Biological Process GO:0016042 lipid catabolic process

Reference

[1] Chen Y, Sun W, Sun Z, Zhao H, Wu T et al.. Effect of electroacupuncture on hippocampal protein lactylation in a rat model of vascular dementia.. Front Neurol 16:1629474. 2025. PMID: 40963935.