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Overview

Uniprot ID	Q99MN9
Protein Name	Propionyl-CoA carboxylase beta chain, mitochondrial
Gene Name	Pccb
Organism	Mus musculus

Kla Sites from experimental identification

Position	Flanking peptide
101	DFGMAADKNKFPGDS
103	GMAADKNKFPGDSVV

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 MAAAIRIRAV 20 AAGARLSVLN 30 CGLGITTRGL 40 CSQPVSVKER 50 IDNKRHAALL 60 GGGQRRIDAQ 70 HKRGKLTARE 80 RISLLLDPGS 90 FMESDMFVEH 100 RCADFGMAAD 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 EVVKSVTNED 250 VTQEQLGGAK 260 THTTVSGVAH 270 RAFDNDVDAL 280 CNLREFFNFL 290 PLSSQDPAPI 300 RECHDPSDRL 310 VPELDTVVPL 320 ESSKAYNMLD 330 IIHAVIDERE 340 FFEIMPSYAK 350 NIVVGFARMN 360 GRTVGIVGNQ 370 PNVASGCLDI 380 NSSVKGARFV 390 RFCDAFNIPL 400 ITFVDVPGFL 410 PGTAQEYGGI 420 IRHGAKLLYA 430 FAEATVPKIT 440 VITRKAYGGA 450 YDVMSSKHLL 460 GDTNYAWPTA 470 EIAVMGAKGA 480 VEIIFKGHQD 490 VEAAQAEYVE 500 KFANPFPAAV 510 RGFVDDIIQP 520 SSTRARICCD 530 LEVLASKKVH 540 RPWRKHANIP 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:0009062	fatty acid catabolic process

Reference

[1] Zhuo W, Zhang M, Tan J, Gao Y, Wang Y et al.. Lysine lactylation analysis of proteins in the heart of the Kawasaki disease mouse model.. Front Cell Dev Biol 13:1550220. 2025. PMID: 40114965.

[2] Wu D, Tang Y, Li X, Xiong S, Zhang Z et al.. Characterization of protein lactylation in healthy and ischemic mouse hearts.. Front Cardiovasc Med 12:1644886. 2025. PMID: 41089239.