Search Results

Overview

Uniprot IDA0A8I6A8G9
Protein NamePropionyl-CoA carboxylase alpha chain, mitochondrial
Gene NamePcca
OrganismRattus norvegicus

Kla Sites from experimental identification

Position Flanking peptide
199 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 VSKAGLAFFC 50 LPWTLHRAPV 60 YSQQCLVVSR 70 SLSSVEYEPK 80 EKTFDKILIA 90 NRGEIACRVI 100 KTCRKMGIRT 110 VAIHSDVDAS 120 SVHVKMADEA 130 VCVGPAPTSK 140 SYLNMDAIME 150 AIKKTGAQAV 160 HPGYGFLSEN 170 KEFAKCLAAE 180 DVTFIGPDTH 190 AIQAMGDKIE 200 SKLLAKRAKV 210 NTIPGFDGVV 220 KDADEAVRIA 230 REIGYPVMIK 240 ASAGGGGKGM 250 RIAWDDEETR 260 DGFRFSSQEA 270 ASSFGDDRLL 280 IEKFIDNPRH 290 IEIQVLGDKH 300 GNALWLNERE 310 CSIQRRNQKV 320 VEEAPSIFLD 330 PETRRAMGEQ 340 AVALAKAVKY 350 SSAGTVEFLV 360 DSQKNFYFLE 370 MNTRLQVEHP 380 VTECITGLDL 390 VQEMILVAKG 400 YPLRHKQEDI 410 PISGWAVECR 420 VYAEDPYKSF 430 GLPSIGRLSQ 440 YQEPIHLPGV 450 RVDSGIQPGS 460 DISIYYDPMI 470 SKLVTYGSDR 480 AEALKRMEDA 490 LDSYVIRGVT 500 HNIPLLREVI 510 INTRFVKGDI 520 STKFLSDVYP 530 DGFKGHMLTP 540 SERDQLLAIA 550 SSLFVASQLR 560 AQRFQEHSRV 570 PVIRPDVAKW 580 ELSVKLHDED 590 HTVVASNNGP 600 TFNVEVDGSK 610 LNVTSTWNLA 620 SPLLSVNVDG 630 TQRTVQCLSR 640 DAGGNMSIQF 650 LGTVYKVHIL 660 TKLAAELNKF 670 MLEKVPKDTS 680 STLCSPMPGV 690 VVAVSVKPGD 700 MVAEGQEICV 710 IEAMKMQNSM 720 TAGKMGKVKL 730 VHCKAGDTVG EGDLLVELE

Gene Ontology

Classification GO ID Description
Cellular Component GO:0005759 mitochondrial matrix
Molecular Function GO:0005524 ATP binding
Molecular Function GO:0019899 enzyme 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
Biological Process GO:1901290 succinyl-CoA biosynthetic 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.