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Overview

Uniprot IDP07633
Protein NamePropionyl-CoA carboxylase beta chain, mitochondrial
Gene NamePccb
OrganismRattus norvegicus

Kla Sites from experimental identification

Position Flanking peptide
103 GMAAEKNKFPGDSVV
250 QEQLGGAKTHTTVSG
476 GAVEIIFKGHEDVEA

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 (By similarity). 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 (By similarity). Other alternative minor substrates include (2E)-butenoyl-CoA/crotonoyl-CoA (By similarity)

Protein Sequence

10 MAAVIRIRAM 20 AAGTRLRVLN 30 CGLGTTIRSL 40 CSQPVSVNER 50 IENKRHAALL 60 GGGQRRIDAQ 70 HKRGKLTARE 80 RISLLLDPGS 90 FLESDMFVEH 100 RCADFGMAAE 110 KNKFPGDSVV 120 TGRGRINGRL 130 VYVFSQDFTV 140 FGGSLSGAHA 150 QKICKIMDQA 160 ITVGAPVIGL 170 NDSGGARIQE 180 GVESLAGYAD 190 IFLRNVTASG 200 VIPQISLIMG 210 PCAGGAVYSP 220 ALTDFTFMVK 230 DTSYLFITGP 240 EFVKSVTNED 250 VTQEQLGGAK 260 THTTVSGVAH 270 RAFDNDVDAL 280 CNLREFLNFL 290 PLSNQDPASI 300 RECHDPSDRL 310 VPELDTVVPL 320 ESSKAYNMLD 330 IIHAVIDERE 340 FFEIMPNYAK 350 NIVIGFARMN 360 GRTVGIVGNQ 370 PNVASGCLDI 380 NSSVKGARFV 390 RFCDAFSIPL 400 ITFVDVPGFL 410 PGTAQEYGGI 420 IRHGAKLLYA 430 FAEATVPKIT 440 VITRKAYGGA 450 YDVMSSKHLL 460 GDTNYAWPTA 470 EIAVMGAKGA 480 VEIIFKGHED 490 VEAAQAEYVE 500 KFANPFPAAV 510 RGFVDDIIQP 520 SSTRARICCD 530 LEVLASKKVH 540 RPWRKHANVP L

Gene Ontology

Classification GO ID Description
Cellular Component GO:1902494 catalytic complex
Cellular Component GO:0005759 mitochondrial matrix
Cellular Component GO:0005739 mitochondrion
Molecular Function GO:0005524 ATP binding
Molecular Function GO:0004658 propionyl-CoA carboxylase activity
Biological Process GO:0009063 amino acid catabolic process
Biological Process GO:0009062 fatty acid 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.