CPLM 1.0 - Compendium of Protein Lysine Modification
TagContent
CPLM ID CPLM-019969
UniProt Accession
Genbank Protein ID
Genbank Nucleotide ID
Protein Name
 Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial 
Protein Synonyms/Alias
 MCCase subunit alpha; 3-methylcrotonyl-CoA carboxylase 1; 3-methylcrotonyl-CoA carboxylase biotin-containing subunit; 3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha 
Gene Name
 Mccc1 
Gene Synonyms/Alias
 Mcca 
Created Date
 July 27, 2013 
Organism
 Mus musculus (Mouse) 
NCBI Taxa ID
 10090 
Lysine Modification
Position
Peptide
Type
References
180VVEGYHGKDQSDQCLacetylation[1]
193CLREHAGKIGYPVMIacetylation[1]
244DDAMLIEKFVDTPRHacetylation[1]
363LRIAAGEKIPLSQEEacetylation[1]
490DFIPQHHKDLLPSHSacetylation[2, 3]
501PSHSTIAKESVCQAAacetylation[2]
514AALGLILKEKEMTSAacetylation[1, 2, 3]
577YDMQIDNKSFRVLGDacetylation[1, 2, 3, 4, 5]
577YDMQIDNKSFRVLGDsuccinylation[5]
659TIEKVFVKAGDRVKAacetylation[1, 2]
692PKDGRIKKVFFSEGAacetylation[2]
717FEEEESDK*******acetylation[3]
Reference
 [1] Calorie restriction and SIRT3 trigger global reprogramming of the mitochondrial protein acetylome.
 Hebert AS, Dittenhafer-Reed KE, Yu W, Bailey DJ, Selen ES, Boersma MD, Carson JJ, Tonelli M, Balloon AJ, Higbee AJ, Westphall MS, Pagliarini DJ, Prolla TA, Assadi-Porter F, Roy S, Denu JM, Coon JJ.
 Mol Cell. 2013 Jan 10;49(1):186-99. [PMID: 23201123]
 [2] Label-free quantitative proteomics of the lysine acetylome in mitochondria identifies substrates of SIRT3 in metabolic pathways.
 Rardin MJ, Newman JC, Held JM, Cusack MP, Sorensen DJ, Li B, Schilling B, Mooney SD, Kahn CR, Verdin E, Gibson BW.
 Proc Natl Acad Sci U S A. 2013 Apr 16;110(16):6601-6. [PMID: 23576753]
 [3] Quantification of mitochondrial acetylation dynamics highlights prominent sites of metabolic regulation.
 Still AJ, Floyd BJ, Hebert AS, Bingman CA, Carson JJ, Gunderson DR, Dolan BK, Grimsrud PA, Dittenhafer-Reed KE, Stapleton DS, Keller MP, Westphall MS, Denu JM, Attie AD, Coon JJ, Pagliarini DJ.
 J Biol Chem. 2013 Jul 17;. [PMID: 23864654]
 [4] Quantitative assessment of the impact of the gut microbiota on lysine epsilon-acetylation of host proteins using gnotobiotic mice.
 Simon GM, Cheng J, Gordon JI.
 Proc Natl Acad Sci U S A. 2012 Jul 10;109(28):11133-8. [PMID: 22733758]
 [5] SIRT5-Mediated Lysine Desuccinylation Impacts Diverse Metabolic Pathways.
 Park J, Chen Y, Tishkoff DX, Peng C, Tan M, Dai L, Xie Z, Zhang Y, Zwaans BM, Skinner ME, Lombard DB, Zhao Y.
 Mol Cell. 2013 Jun 27;50(6):919-30. [PMID: 23806337
Functional Description
 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 (By similarity). 
Sequence Annotation
 DOMAIN 45 490 Biotin carboxylation.
 DOMAIN 163 360 ATP-grasp.
 DOMAIN 630 710 Biotinyl-binding.
 ACT_SITE 335 335 By similarity.
 BINDING 159 159 ATP (By similarity).
 BINDING 243 243 ATP (By similarity).
 BINDING 278 278 ATP (By similarity).
 MOD_RES 677 677 N6-biotinyllysine (By similarity).  
Keyword
 ATP-binding; Biotin; Complete proteome; Ligase; Mitochondrion; Nucleotide-binding; Reference proteome; Transit peptide. 
Sequence Source
 UniProt (SWISSPROT/TrEMBL); GenBank; EMBL 
Protein Length
 717 AA 
Protein Sequence
MAAAALLAAV DRNQLRRVPI LLLQPREWAW KLRTMKYGTT PGGSITKVLI ANRGEIACRV 60
IRTAKKMGVQ SVAVYSEADR NSMHVDMADE AYSIGPAPSQ QSYLAMEKII QVAKSSAAQA 120
IHPGYGFLSE NMEFAELCKQ EGIIFIGPPS SAIRDMGIKS TSKSIMAAAG VPVVEGYHGK 180
DQSDQCLREH AGKIGYPVMI KAVRGGGGKG MRIVRSEREF QEQLESARRE AKKSFNDDAM 240
LIEKFVDTPR HVEVQVFGDH HGNAVYLFER DCSVQRRHQK IIEEAPAPGI NPEVRRKLGE 300
AAVRAAKAVK YVGAGTVEFI MDSRHNFYFM EMNTRLQVEH PVTEMITGTD LVEWQLRIAA 360
GEKIPLSQEE IPLQGHAFEA RIYAEDPDNN FMPGAGPLVH LSTPSADMST RIETGVRQGD 420
EVSVHYDPMI AKLVVWASDR QSALSKLRYC LHQYNIVGLR SNVDFLLRLS GHPEFEAGNV 480
HTDFIPQHHK DLLPSHSTIA KESVCQAALG LILKEKEMTS AFKLHTQDQF SPFSFSSGRR 540
LNISYTRNMT LRSGKSDIVI AVTYNRDGSY DMQIDNKSFR VLGDLSSEDG CTYLKSSING 600
VARKSKFILL DNTVHLFSME GSIEVGIPVP KYLSPVSAEG AQGGTIAPMT GTIEKVFVKA 660
GDRVKAGDSL MVMIAMKMEH TIKAPKDGRI KKVFFSEGAQ ANRHAPLVEF EEEESDK 717 
Gene Ontology
 GO:0005743; C:mitochondrial inner membrane; IDA:MGI.
 GO:0005759; C:mitochondrial matrix; IEA:UniProtKB-SubCell.
 GO:0005524; F:ATP binding; IEA:UniProtKB-KW.
 GO:0004075; F:biotin carboxylase activity; IEA:InterPro.
 GO:0046872; F:metal ion binding; IEA:InterPro.
 GO:0004485; F:methylcrotonoyl-CoA carboxylase activity; ISS:UniProtKB.
 GO:0006552; P:leucine catabolic process; IEA:UniProtKB-UniPathway. 
Interpro
 IPR011761; ATP-grasp.
 IPR013815; ATP_grasp_subdomain_1.
 IPR013816; ATP_grasp_subdomain_2.
 IPR001882; Biotin_BS.
 IPR011764; Biotin_carboxylation_dom.
 IPR005482; Biotin_COase_C.
 IPR000089; Biotin_lipoyl.
 IPR005481; CarbamoylP_synth_lsu_N.
 IPR005479; CbamoylP_synth_lsu-like_ATP-bd.
 IPR016185; PreATP-grasp_dom.
 IPR011054; Rudment_hybrid_motif.
 IPR011053; Single_hybrid_motif. 
Pfam
 PF02785; Biotin_carb_C
 PF00364; Biotin_lipoyl
 PF00289; CPSase_L_chain
 PF02786; CPSase_L_D2 
SMART
 SM00878; Biotin_carb_C 
PROSITE
 PS50975; ATP_GRASP
 PS50979; BC
 PS00188; BIOTIN
 PS50968; BIOTINYL_LIPOYL
 PS00867; CPSASE_2 
PRINTS