Search Results

Overview

Uniprot ID	Q99MR8
Protein Name	Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial
Gene Name	Mccc1
Organism	Mus musculus

Kla Sites from experimental identification

Position	Flanking peptide
180	VVEGYHGKDQSDQCL
193	CLREHAGKIGYPVMI
233	SARREAKKSFNDDAM
490	DFIPQHHKDLLPSHS
659	TIEKVFVKAGDRVKA
692	PKDGRIKKVFFSEGA

Function

Biotin-attachment subunit of the 3-methylcrotonyl-CoA carboxylase, an enzyme that catalyzes the conversion of 3-methylcrotonyl-CoA to 3-methylglutaconyl-CoA, a critical step for leucine and isovaleric acid catabolism

Protein Sequence

10 MAAAALLAAV 20 DRNQLRRVPI 30 LLLQPREWAW 40 KLRTMKYGTT 50 PGGSITKVLI 60 ANRGEIACRV 70 IRTAKKMGVQ 80 SVAVYSEADR 90 NSMHVDMADE 100 AYSIGPAPSQ 110 QSYLAMEKII 120 QVAKSSAAQA 130 IHPGYGFLSE 140 NMEFAELCKQ 150 EGIIFIGPPS 160 SAIRDMGIKS 170 TSKSIMAAAG 180 VPVVEGYHGK 190 DQSDQCLREH 200 AGKIGYPVMI 210 KAVRGGGGKG 220 MRIVRSEREF 230 QEQLESARRE 240 AKKSFNDDAM 250 LIEKFVDTPR 260 HVEVQVFGDH 270 HGNAVYLFER 280 DCSVQRRHQK 290 IIEEAPAPGI 300 NPEVRRKLGE 310 AAVRAAKAVK 320 YVGAGTVEFI 330 MDSRHNFYFM 340 EMNTRLQVEH 350 PVTEMITGTD 360 LVEWQLRIAA 370 GEKIPLSQEE 380 IPLQGHAFEA 390 RIYAEDPDNN 400 FMPGAGPLVH 410 LSTPSADMST 420 RIETGVRQGD 430 EVSVHYDPMI 440 AKLVVWASDR 450 QSALSKLRYC 460 LHQYNIVGLR 470 SNVDFLLRLS 480 GHPEFEAGNV 490 HTDFIPQHHK 500 DLLPSHSTIA 510 KESVCQAALG 520 LILKEKEMTS 530 AFKLHTQDQF 540 SPFSFSSGRR 550 LNISYTRNMT 560 LRSGKSDIVI 570 AVTYNRDGSY 580 DMQIDNKSFR 590 VLGDLSSEDG 600 CTYLKSSING 610 VARKSKFILL 620 DNTVHLFSME 630 GSIEVGIPVP 640 KYLSPVSAEG 650 AQGGTIAPMT 660 GTIEKVFVKA 670 GDRVKAGDSL 680 MVMIAMKMEH 690 TIKAPKDGRI 700 KKVFFSEGAQ 710 ANRHAPLVEF EEEESDK

Gene Ontology

Classification	GO ID	Description
Cellular Component	GO:1905202	methylcrotonoyl-CoA carboxylase complex
Cellular Component	GO:0005743	mitochondrial inner membrane
Cellular Component	GO:0005759	mitochondrial matrix
Cellular Component	GO:0005739	mitochondrion
Molecular Function	GO:0005524	ATP binding
Molecular Function	GO:0046872	metal ion binding
Molecular Function	GO:0004485	methylcrotonoyl-CoA carboxylase activity
Biological Process	GO:0006552	L-leucine 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.