Search Results

Overview

Uniprot ID	Q96RQ3
Protein Name	Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial
Gene Name	MCCC1
Organism	Homo sapiens

Kla Sites from experimental identification

Position	Flanking peptide
213	AVRGGGGKGMRIVRS
237	SARREAKKSFNDDAM
295	EAPAPGIKSEVRKKL
301	IKSEVRKKLGEAAVR

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 MAAASAVSVL 20 LVAAERNRWH 30 RLPSLLLPPR 40 TWVWRQRTMK 50 YTTATGRNIT 60 KVLIANRGEI 70 ACRVMRTAKK 80 LGVQTVAVYS 90 EADRNSMHVD 100 MADEAYSIGP 110 APSQQSYLSM 120 EKIIQVAKTS 130 AAQAIHPGCG 140 FLSENMEFAE 150 LCKQEGIIFI 160 GPPPSAIRDM 170 GIKSTSKSIM 180 AAAGVPVVEG 190 YHGEDQSDQC 200 LKEHARRIGY 210 PVMIKAVRGG 220 GGKGMRIVRS 230 EQEFQEQLES 240 ARREAKKSFN 250 DDAMLIEKFV 260 DTPRHVEVQV 270 FGDHHGNAVY 280 LFERDCSVQR 290 RHQKIIEEAP 300 APGIKSEVRK 310 KLGEAAVRAA 320 KAVNYVGAGT 330 VEFIMDSKHN 340 FCFMEMNTRL 350 QVEHPVTEMI 360 TGTDLVEWQL 370 RIAAGEKIPL 380 SQEEITLQGH 390 AFEARIYAED 400 PSNNFMPVAG 410 PLVHLSTPRA 420 DPSTRIETGV 430 RQGDEVSVHY 440 DPMIAKLVVW 450 AADRQAALTK 460 LRYSLRQYNI 470 VGLHTNIDFL 480 LNLSGHPEFE 490 AGNVHTDFIP 500 QHHKQLLLSR 510 KAAAKESLCQ 520 AALGLILKEK 530 AMTDTFTLQA 540 HDQFSPFSSS 550 SGRRLNISYT 560 RNMTLKDGKN 570 NVAIAVTYNH 580 DGSYSMQIED 590 KTFQVLGNLY 600 SEGDCTYLKC 610 SVNGVASKAK 620 LIILENTIYL 630 FSKEGSIEID 640 IPVPKYLSSV 650 SSQETQGGPL 660 APMTGTIEKV 670 FVKAGDKVKA 680 GDSLMVMIAM 690 KMEHTIKSPK 700 DGTVKKVFYR 710 EGAQANRHTP 720 LVEFEEEESD KRESE

Gene Ontology

Classification	GO ID	Description
Cellular Component	GO:0005829	cytosol
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:0009374	biotin binding
Molecular Function	GO:0004075	biotin carboxylase activity
Molecular Function	GO:0046872	metal ion binding
Molecular Function	GO:0004485	methylcrotonoyl-CoA carboxylase activity
Biological Process	GO:0006768	biotin metabolic process
Biological Process	GO:0009083	branched-chain amino acid catabolic process
Biological Process	GO:0006552	L-leucine catabolic process

Reference

[1] Yang Z, Yan C, Ma J, Peng P, Ren X et al.. Lactylome analysis suggests lactylation-dependent mechanisms of metabolic adaptation in hepatocellular carcinoma.. Nat Metab 5(1):61-79. 2023 Jan. PMID: 36593272.

[2] Hong H, Chen X, Wang H, Gu X, Yuan Y et al.. Global profiling of protein lysine lactylation and potential target modified protein analysis in hepatocellular carcinoma.. Proteomics 23(9):e2200432. 2023 May. PMID: 36625413.

[3] Shi CM, Wang QC, Li XL, Yang YH, Tang XY et al.. Global Profiling of Protein Lactylation in Human Hippocampi.. Proteomics Clin Appl 19(2):e202400061. 2025 Mar. PMID: 39610256.