Search Results

Overview

Uniprot IDQ91ZA3
Protein NamePropionyl-CoA carboxylase alpha chain, mitochondrial
Gene NamePcca
OrganismMus musculus

Kla Sites from experimental identification

Position Flanking peptide
146 YGFLSENKEFAKRLA
184 KLLAKRAKVNTIPGF
381 KGYPLRHKQEDIPIS
492 IINTRFVKGDISTKF
61 PKEKTFDKILIANRG
704 AGKMGKVKLVHCKAG

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 MAGQWVRTVA 20 LLAARRHWRR 30 SSQQQLLGTL 40 KHAPVYSYQC 50 LVVSRSLSSV 60 EYEPKEKTFD 70 KILIANRGEI 80 ACRVIKTCKK 90 MGIKTVAIHS 100 DVDASSVHVK 110 MADEAVCVGP 120 APTSKSYLNM 130 DAIMEAIKKT 140 RAQAVHPGYG 150 FLSENKEFAK 160 RLAAEDVTFI 170 GPDTHAIQAM 180 GDKIESKLLA 190 KRAKVNTIPG 200 FDGVVKDADE 210 AVRIAREIGY 220 PVMIKASAGG 230 GGKGMRIAWD 240 DEETRDGFRF 250 SSQEAASSFG 260 DDRLLIEKFI 270 DNPRHIEIQV 280 LGDKHGNALW 290 LNERECSIQR 300 RNQKVVEEAP 310 SIFLDPETRQ 320 AMGEQAVALA 330 KAVKYSSAGT 340 VEFLVDSQKN 350 FYFLEMNTRL 360 QVEHPVTECI 370 TGLDLVQEMI 380 LVAKGYPLRH 390 KQEDIPISGW 400 AVECRVYAED 410 PYKSFGLPSI 420 GRLSQYQEPI 430 HLPGVRVDSG 440 IQPGSDISIY 450 YDPMISKLVT 460 YGSDRAEALK 470 RMEDALDNYV 480 IRGVTHNIPL 490 LREVIINTRF 500 VKGDISTKFL 510 SDVYPDGFKG 520 HTLTLSERNQ 530 LLAIASSVFV 540 ASQLRAQRFQ 550 EHSRVPVIRP 560 DVAKWELSVK 570 LHDEDHTVVA 580 SNNGPAFTVE 590 VDGSKLNVTS 600 TWNLASPLLS 610 VNVDGTQRTV 620 QCLSREAGGN 630 MSIQFLGTVY 640 KVHILTKLAA 650 ELNKFMLEKV 660 PKDTSSTLCS 670 PMPGVVVAVS 680 VKPGDMVAEG 690 QEICVIEAMK 700 MQNSMTAGKM 710 GKVKLVHCKA 720 GDTVGEGDLL VELE

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:0019899 enzyme binding
Molecular Function GO:0046872 metal ion binding
Molecular Function GO:0004658 propionyl-CoA carboxylase activity
Biological Process GO:0016042 lipid catabolic process
Biological Process GO:1901290 succinyl-CoA biosynthetic 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.