Search Results

Overview

Uniprot ID	Q5I0C3
Protein Name	Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial
Gene Name	Mccc1
Organism	Rattus norvegicus

Kla Sites from experimental identification

Position	Flanking peptide
218	MRIIRSEKEFQEQLE
233	SARREAKKSFNDDAM
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 LLLQPREWPW 40 KHRTVKYGTT 50 PGGSITKVLI 60 ANRGEIACRV 70 IRTARKMGVQ 80 SVAVYSEADR 90 NSMHVDMADE 100 AYSIGPAPSQ 110 QSYLAMEKII 120 QVAKSSAAQA 130 IHPGYGFLSE 140 NMEFAEFCKQ 150 EGIIFIGPPS 160 TAIRDMGIKS 170 TSKSIMAAAG 180 VPVVEGYHGN 190 DQSDECLKEH 200 AGKIGYPVMI 210 KAIRGGGGKG 220 MRIIRSEKEF 230 QEQLESARRE 240 AKKSFNDDAM 250 LIEKFVDTPR 260 HVEVQVFGDH 270 HGNAVYLFER 280 DCSVQRRHQK 290 IIEEAPAPGI 300 DPEVRRRLGE 310 AAVRAAKAVN 320 YVGAGTVEFI 330 MDSKHNFYFM 340 EMNTRLQVEH 350 PVTEMITGTD 360 LVEWQLRIAA 370 GEKIPLSQEE 380 IPLQGHAFEA 390 RIYAEDPDNN 400 FMPGAGPLVH 410 LSTPPPDMST 420 RIETGVRQGD 430 EVSVHYDPMI 440 AKLVVWASDR 450 QSALSKLRYS 460 LHQYNIVGLR 470 TNVDFLLRLS 480 GHSEFEAGNV 490 HTDFIPQHHK 500 DLLPTHSTIA 510 KESVCQAALG 520 LILKEKEMTS 530 AFKLHTQDQF 540 SPFSFSSGRR 550 LNISYTRNMT 560 LRSGKNDIII 570 AVTYNRDGSY 580 DMQIENKLFR 590 VLGDLSNEDG 600 YTYLKSSVNG 610 VASKSKFILL 620 DNTIYLFSME 630 GSIEVGIPVP 640 KYLSPVSAEG 650 TQGGTIAPMT 660 GTIEKVFVKA 670 GDRVKAGDAL 680 MVMIAMKMEH 690 TIKAPKDGRI 700 KKVFFSEGAQ 710 ANRHAPLVEF EEEEV

Gene Ontology

Classification	GO ID	Description
Cellular Component	GO:1905202	methylcrotonoyl-CoA carboxylase complex
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] 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.