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Overview

Uniprot IDP16332
Protein NameMethylmalonyl-CoA mutase, mitochondrial
Gene NameMmut
OrganismMus musculus

Kla Sites from experimental identification

Position Flanking peptide
210 EQGVPKEKLTGTIQN
593 RQEFGESKEITSAIK
600 KEITSAIKRVNKFME
604 SAIKRVNKFMEREGR

Function

Catalyzes the reversible isomerization of methylmalonyl-CoA (MMCoA) (generated from branched-chain amino acid metabolism and degradation of dietary odd chain fatty acids and cholesterol) to succinyl-CoA (3-carboxypropionyl-CoA), a key intermediate of the tricarboxylic acid cycle

Protein Sequence

10 MLRAKNQLFL 20 LSPHYLKQLN 30 IPSASRWKRL 40 LHQQQPLHPE 50 WAVLAKKQLK 60 GKNPEDLIWH 70 TPEGISIKPL 80 YSRADTLDLP 90 EELPGVKPFT 100 RGPYPTMYTY 110 RPWTIRQYAG 120 FSTVEESNKF 130 YKDNIKAGQQ 140 GLSVAFDLAT 150 HRGYDSDNPR 160 VRGDVGMAGV 170 AIDTVEDTKI 180 LFDGIPLEKM 190 SVSMTMNGAV 200 IPVLATFIVT 210 GEEQGVPKEK 220 LTGTIQNDIL 230 KEFMVRNTYI 240 FPPEPSMKII 250 ADIFQYTAQH 260 MPKFNSISIS 270 GYHMQEAGAD 280 AILELAYTIA 290 DGLEYCRTGL 300 QAGLTIDEFA 310 PRLSFFWGIG 320 MNFYMEIAKM 330 RAGRRLWAHL 340 IEKMFQPKNS 350 KSLLLRAHCQ 360 TSGWSLTEQD 370 PYNNIVRTAI 380 EAMAAVFGGT 390 QSLHTNSFDE 400 ALGLPTVKSA 410 RIARNTQIII 420 QEESGIPKVA 430 DPWGGSYMME 440 SLTNDVYEAA 450 LKLIYEVEEM 460 GGMAKAVAEG 470 IPKLRIEECA 480 ARRQARIDSG 490 SEVIVGVNKY 500 QLEKEDSVEV 510 LAIDNTSVRK 520 KQIEKLKKIK 530 SSRDQALAEQ 540 CLSALTQCAA 550 SGDGNILALA 560 VDAARARCTV 570 GEITDALKKV 580 FGEHKANDRM 590 VSGAYRQEFG 600 ESKEITSAIK 610 RVNKFMEREG 620 RRPRLLVAKM 630 GQDGHDRGAK 640 VIATGFADLG 650 FDVDIGPLFQ 660 TPREVAQQAV 670 DADVHAVGVS 680 TLAAGHKTLV 690 PELIKELTAL 700 GRPDILVMCG 710 GVIPPQDYEF 720 LYEVGVSNVF 730 GPGTRIPRAA 740 VQVLDDIEKC LAEKQQSV

Gene Ontology

Classification GO ID Description
Cellular Component GO:0005737 cytoplasm
Cellular Component GO:0005759 mitochondrial matrix
Cellular Component GO:0005739 mitochondrion
Molecular Function GO:0031419 cobalamin binding
Molecular Function GO:0003924 GTPase activity
Molecular Function GO:0042802 identical protein binding
Molecular Function GO:0046872 metal ion binding
Molecular Function GO:0004494 methylmalonyl-CoA mutase activity
Molecular Function GO:0072341 modified amino acid binding
Molecular Function GO:0042803 protein homodimerization activity
Biological Process GO:0050667 homocysteine metabolic process
Biological Process GO:0043547 positive regulation of GTPase activity
Biological Process GO:0009791 post-embryonic development
Biological Process GO:0019678 propionate metabolic process, methylmalonyl pathway
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.