LOCUS       NP_076133                717 aa            linear   ROD 02-MAY-2024
DEFINITION  methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial [Mus
            musculus].
ACCESSION   NP_076133 XP_918268
VERSION     NP_076133.3
DBSOURCE    REFSEQ: accession NM_023644.5
KEYWORDS    RefSeq; RefSeq Select.
SOURCE      Mus musculus (house mouse)
  ORGANISM  Mus musculus
            Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi;
            Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha;
            Muroidea; Muridae; Murinae; Mus; Mus.
REFERENCE   1  (residues 1 to 717)
  AUTHORS   Adams,D.J., Barlas,B., McIntyre,R.E., Salguero,I., van der
            Weyden,L., Barros,A., Vicente,J.R., Karimpour,N., Haider,A.,
            Ranzani,M., Turner,G., Thompson,N.A., Harle,V., Olvera-Leon,R.,
            Robles-Espinoza,C.D., Speak,A.O., Geisler,N., Weninger,W.J.,
            Geyer,S.H., Hewinson,J., Karp,N.A., Fu,B., Yang,F., Kozik,Z.,
            Choudhary,J., Yu,L., van Ruiten,M.S., Rowland,B.D., Lelliott,C.J.,
            Del Castillo Velasco-Herrera,M., Verstraten,R., Bruckner,L.,
            Henssen,A.G., Rooimans,M.A., de Lange,J., Mohun,T.J., Arends,M.J.,
            Kentistou,K.A., Coelho,P.A., Zhao,Y., Zecchini,H., Perry,J.R.B.,
            Jackson,S.P. and Balmus,G.
  CONSRTM   Sanger Mouse Genetics Project
  TITLE     Genetic determinants of micronucleus formation in vivo
  JOURNAL   Nature 627 (8002), 130-136 (2024)
   PUBMED   38355793
REFERENCE   2  (residues 1 to 717)
  AUTHORS   Bailey,P.J., Klos,J.M., Andersson,E., Karlen,M., Kallstrom,M.,
            Ponjavic,J., Muhr,J., Lenhard,B., Sandelin,A. and Ericson,J.
  TITLE     A global genomic transcriptional code associated with CNS-expressed
            genes
  JOURNAL   Exp Cell Res 312 (16), 3108-3119 (2006)
   PUBMED   16919269
REFERENCE   3  (residues 1 to 717)
  AUTHORS   Mootha,V.K., Bunkenborg,J., Olsen,J.V., Hjerrild,M.,
            Wisniewski,J.R., Stahl,E., Bolouri,M.S., Ray,H.N., Sihag,S.,
            Kamal,M., Patterson,N., Lander,E.S. and Mann,M.
  TITLE     Integrated analysis of protein composition, tissue diversity, and
            gene regulation in mouse mitochondria
  JOURNAL   Cell 115 (5), 629-640 (2003)
   PUBMED   14651853
REFERENCE   4  (residues 1 to 717)
  AUTHORS   Da Cruz,S., Xenarios,I., Langridge,J., Vilbois,F., Parone,P.A. and
            Martinou,J.C.
  TITLE     Proteomic analysis of the mouse liver mitochondrial inner membrane
  JOURNAL   J Biol Chem 278 (42), 41566-41571 (2003)
   PUBMED   12865426
REFERENCE   5  (residues 1 to 717)
  AUTHORS   Obata,K., Fukuda,T., Morishita,R., Abe,S., Asakawa,S.,
            Yamaguchi,S., Yoshino,M., Ihara,K., Murayama,K., Shigemoto,K.,
            Shimizu,N. and Kondo,I.
  TITLE     Human biotin-containing subunit of 3-methylcrotonyl-CoA carboxylase
            gene (MCCA): cDNA sequence, genomic organization, localization to
            chromosomal band 3q27, and expression
  JOURNAL   Genomics 72 (2), 145-152 (2001)
   PUBMED   11401427
REFERENCE   6  (residues 1 to 717)
  AUTHORS   Baumgartner,M.R., Almashanu,S., Suormala,T., Obie,C., Cole,R.N.,
            Packman,S., Baumgartner,E.R. and Valle,D.
  TITLE     The molecular basis of human 3-methylcrotonyl-CoA carboxylase
            deficiency
  JOURNAL   J Clin Invest 107 (4), 495-504 (2001)
   PUBMED   11181649
REFERENCE   7  (residues 1 to 717)
  AUTHORS   Gallardo,M.E., Desviat,L.R., Rodriguez,J.M., Esparza-Gordillo,J.,
            Perez-Cerda,C., Perez,B., Rodriguez-Pombo,P., Criado,O., Sanz,R.,
            Morton,D.H., Gibson,K.M., Le,T.P., Ribes,A., de Cordoba,S.R.,
            Ugarte,M. and Penalva,M.A.
  TITLE     The molecular basis of 3-methylcrotonylglycinuria, a disorder of
            leucine catabolism
  JOURNAL   Am J Hum Genet 68 (2), 334-346 (2001)
   PUBMED   11170888
COMMENT     VALIDATED REFSEQ: This record has undergone validation or
            preliminary review. The reference sequence was derived from
            AC116677.11.
            
            On Apr 25, 2008 this sequence version replaced NP_076133.2.
            
            ##Evidence-Data-START##
            Transcript exon combination :: AF310338.1, AK031072.1 [ECO:0000332]
            RNAseq introns              :: mixed sample support SAMN00849374,
                                           SAMN00849375 [ECO:0006172]
            ##Evidence-Data-END##
            
            ##RefSeq-Attributes-START##
            gene product(s) localized to mito. :: reported by MitoCarta
            RefSeq Select criteria             :: based on single
                                                  protein-coding transcript
            ##RefSeq-Attributes-END##
FEATURES             Location/Qualifiers
     source          1..717
                     /organism="Mus musculus"
                     /strain="C57BL/6"
                     /db_xref="taxon:10090"
                     /chromosome="3"
                     /map="3 17.41 cM"
     Protein         1..717
                     /product="methylcrotonoyl-CoA carboxylase subunit alpha,
                     mitochondrial"
                     /EC_number="6.4.1.4"
                     /note="3-methylcrotonyl-CoA carboxylase biotin-containing
                     subunit; MCCase subunit alpha; 3-methylcrotonyl-CoA
                     carboxylase 1; 3-methylcrotonyl-CoA:carbon dioxide ligase
                     subunit alpha"
                     /calculated_mol_wt=74941
     transit_peptide 1..38
                     /note="Mitochondrion.
                     /evidence=ECO:0000250|UniProtKB:Q96RQ3; propagated from
                     UniProtKB/Swiss-Prot (Q99MR8.2)"
                     /calculated_mol_wt=4421
     mat_peptide     39..717
                     /product="Methylcrotonoyl-CoA carboxylase subunit alpha,
                     mitochondrial. /id=PRO_0000002834"
                     /note="propagated from UniProtKB/Swiss-Prot (Q99MR8.2)"
                     /calculated_mol_wt=74941
     Region          45..495
                     /region_name="PccA"
                     /note="Acetyl/propionyl-CoA carboxylase, alpha subunit
                     [Lipid transport and metabolism]; COG4770"
                     /db_xref="CDD:443802"
     Site            180
                     /site_type="acetylation"
                     /note="N6-acetyllysine.
                     /evidence=ECO:0007744|PubMed:23576753; propagated from
                     UniProtKB/Swiss-Prot (Q99MR8.2)"
     Site            193
                     /site_type="acetylation"
                     /note="N6-acetyllysine.
                     /evidence=ECO:0007744|PubMed:23576753; propagated from
                     UniProtKB/Swiss-Prot (Q99MR8.2)"
     Site            233
                     /site_type="acetylation"
                     /note="N6-acetyllysine.
                     /evidence=ECO:0007744|PubMed:23576753; propagated from
                     UniProtKB/Swiss-Prot (Q99MR8.2)"
     Site            490
                     /site_type="acetylation"
                     /note="N6-acetyllysine.
                     /evidence=ECO:0007744|PubMed:23576753; propagated from
                     UniProtKB/Swiss-Prot (Q99MR8.2)"
     Site            577
                     /site_type="acetylation"
                     /note="N6-acetyllysine, alternate.
                     /evidence=ECO:0007744|PubMed:23576753; propagated from
                     UniProtKB/Swiss-Prot (Q99MR8.2)"
     Region          647..710
                     /region_name="biotinyl_domain"
                     /note="The biotinyl-domain or biotin carboxyl carrier
                     protein (BCCP) domain is present in all biotin-dependent
                     enzymes, such as acetyl-CoA carboxylase, pyruvate
                     carboxylase, propionyl-CoA carboxylase, methylcrotonyl-CoA
                     carboxylase, geranyl-CoA carboxylase; cd06850"
                     /db_xref="CDD:133459"
     Site            order(667,676..678,685)
                     /site_type="active"
                     /note="carboxyltransferase (CT) interaction site [active]"
                     /db_xref="CDD:133459"
     Site            677
                     /site_type="other"
                     /note="biotinylation site [posttranslational
                     modification]"
                     /db_xref="CDD:133459"
     CDS             1..717
                     /gene="Mccc1"
                     /gene_synonym="1810045E08Rik; 2310058B18Rik; Mcca;
                     MCCalpha"
                     /coded_by="NM_023644.5:112..2265"
                     /db_xref="CCDS:CCDS17307.1"
                     /db_xref="GeneID:72039"
                     /db_xref="MGI:MGI:1919289"
ORIGIN      
        1 maaaallaav drnqlrrvpi lllqprewaw klrtmkygtt pggsitkvli anrgeiacrv
       61 irtakkmgvq svavyseadr nsmhvdmade aysigpapsq qsylamekii qvakssaaqa
      121 ihpgygflse nmefaelckq egiifigpps sairdmgiks tsksimaaag vpvvegyhgk
      181 dqsdqclreh agkigypvmi kavrggggkg mrivrseref qeqlesarre akksfnddam
      241 liekfvdtpr hvevqvfgdh hgnavylfer dcsvqrrhqk iieeapapgi npevrrklge
      301 aavraakavk yvgagtvefi mdsrhnfyfm emntrlqveh pvtemitgtd lvewqlriaa
      361 gekiplsqee iplqghafea riyaedpdnn fmpgagplvh lstpsadmst rietgvrqgd
      421 evsvhydpmi aklvvwasdr qsalsklryc lhqynivglr snvdfllrls ghpefeagnv
      481 htdfipqhhk dllpshstia kesvcqaalg lilkekemts afklhtqdqf spfsfssgrr
      541 lnisytrnmt lrsgksdivi avtynrdgsy dmqidnksfr vlgdlssedg ctylkssing
      601 varkskfill dntvhlfsme gsievgipvp kylspvsaeg aqggtiapmt gtiekvfvka
      661 gdrvkagdsl mvmiamkmeh tikapkdgri kkvffsegaq anrhaplvef eeeesdk
//
