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    Mccc2 methylcrotonyl-CoA carboxylase subunit 2 [ Rattus norvegicus (Norway rat) ]

    Gene ID: 361884, updated on 9-Dec-2024

    Summary

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    Official Symbol
    Mccc2provided by RGD
    Official Full Name
    methylcrotonyl-CoA carboxylase subunit 2provided by RGD
    Primary source
    RGD:1310279
    See related
    EnsemblRapid:ENSRNOG00000017752 AllianceGenome:RGD:1310279
    Gene type
    protein coding
    RefSeq status
    PROVISIONAL
    Organism
    Rattus norvegicus
    Lineage
    Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae; Murinae; Rattus
    Summary
    Contributes to methylcrotonoyl-CoA carboxylase activity. Involved in coenzyme A metabolic process. Predicted to be located in mitochondrial matrix. Predicted to be part of methylcrotonoyl-CoA carboxylase complex. Predicted to be active in mitochondrion. Human ortholog(s) of this gene implicated in 3-methylcrotonyl-CoA carboxylase 2 deficiency and mitochondrial metabolism disease. Orthologous to human MCCC2 (methylcrotonyl-CoA carboxylase subunit 2). [provided by Alliance of Genome Resources, Dec 2024]
    Expression
    Biased expression in Kidney (RPKM 463.7), Heart (RPKM 405.5) and 9 other tissues See more
    Orthologs
    human mouse all
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    Genomic context

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    See Mccc2 in Genome Data Viewer
    Location:
    2q12
    Exon count:
    18
    Annotation release Status Assembly Chr Location
    RS_2024_02 current GRCr8 (GCF_036323735.1) 2 NC_086020.1 (33039044..33110092, complement)
    RS_2023_06 previous assembly mRatBN7.2 (GCF_015227675.2) 2 NC_051337.1 (31304927..31375978, complement)
    106 previous assembly Rnor_6.0 (GCF_000001895.5) 2 NC_005101.4 (30175017..30246028, complement)

    Chromosome 2 - NC_086020.1Genomic Context describing neighboring genes Neighboring gene ubiquitin conjugating enzyme E2 V1, pseudogene 20 Neighboring gene CART prepropeptide Neighboring gene BDP1 general transcription factor IIIB subunit Neighboring gene small EDRK-rich factor 1

    Genomic regions, transcripts, and products

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    Go to nucleotide: Graphics FASTA GenBank

    Expression

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    • Project title: A rat RNA-Seq transcriptomic BodyMap across 11 organs and 4 developmental stages
    • Description: 320 RNA samples isolated from 11 organs (adrenal gland, brain, heart, kidney, liver, lung, muscle, spleen, thymus, and testes or uterus) from both sexes of Fischer 344 rats across four developmental stages (2-, 6-, 21-, and 104-weeks-old)
    • BioProject: PRJNA238328
    • Publication: PMID 24510058
    • Analysis date: Mon Jun 6 17:44:12 2016

    Bibliography

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    Related articles in PubMed

    1. Simultaneous preparation of the three biotin-containing mitochondrial carboxylases from rat liver. Oei J, et al. Biochim Biophys Acta, 1985 May 29. PMID 3995077
    2. Expression, purification, characterization of human 3-methylcrotonyl-CoA carboxylase (MCCC). Chu CH, et al. Protein Expr Purif, 2007 Jun. PMID 17360195
    3. The molecular basis of 3-methylcrotonylglycinuria, a disorder of leucine catabolism. Gallardo ME, et al. Am J Hum Genet, 2001 Feb. PMID 11170888, Free PMC Article
    4. Mitochondrial targeting signals and mature peptides of 3-methylcrotonyl-CoA carboxylase. Stadler SC, et al. Biochem Biophys Res Commun, 2005 Sep 2. PMID 16023992
    5. Interacting Network of the Gap Junction (GJ) Protein Connexin43 (Cx43) is Modulated by Ischemia and Reperfusion in the Heart. Martins-Marques T, et al. Mol Cell Proteomics, 2015 Nov. PMID 26316108, Free PMC Article

    See all (10) citations in PubMed

    GeneRIFs: Gene References Into Functions

    Pathways from PubChem

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    Interactions

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    Products Interactant Other Gene Complex Source Pubs Description

    General gene information

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    Markers

    Homology

    Gene Ontology Provided by RGD

    Function Evidence Code Pubs
    enables ATP binding IEA
    Inferred from Electronic Annotation
    more info
    		  
    enables ligase activity IEA
    Inferred from Electronic Annotation
    more info
    		  
    contributes_to methylcrotonoyl-CoA carboxylase activity IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  
    contributes_to methylcrotonoyl-CoA carboxylase activity IDA
    Inferred from Direct Assay
    more info
    		 PubMed 
    contributes_to methylcrotonoyl-CoA carboxylase activity ISO
    Inferred from Sequence Orthology
    more info
    		  
    contributes_to methylcrotonoyl-CoA carboxylase activity ISS
    Inferred from Sequence or Structural Similarity
    more info
    		  
    Process Evidence Code Pubs
    involved_in L-leucine catabolic process IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  
    involved_in L-leucine catabolic process IEA
    Inferred from Electronic Annotation
    more info
    		  
    involved_in coenzyme A metabolic process IDA
    Inferred from Direct Assay
    more info
    		 PubMed 
    Component Evidence Code Pubs
    part_of methylcrotonoyl-CoA carboxylase complex IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  
    part_of methylcrotonoyl-CoA carboxylase complex ISO
    Inferred from Sequence Orthology
    more info
    		  
    part_of methylcrotonoyl-CoA carboxylase complex ISS
    Inferred from Sequence or Structural Similarity
    more info
    		  
    located_in mitochondrial matrix ISO
    Inferred from Sequence Orthology
    more info
    		  
    is_active_in mitochondrion IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  
    located_in mitochondrion ISO
    Inferred from Sequence Orthology
    more info
    		  

    General protein information

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    Preferred Names
    methylcrotonoyl-CoA carboxylase beta chain, mitochondrial
    Names
    3-methylcrotonyl-CoA carboxylase 2
    3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit
    3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta
    MCCase subunit beta
    methylcrotonoyl-CoA carboxylase 2 (beta)
    methylcrotonoyl-Coenzyme A carboxylase 2 (beta)
    NP_001012177.1
    XP_038958472.1
    XP_038958474.1

    NCBI Reference Sequences (RefSeq)

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    RefSeqs maintained independently of Annotated Genomes

    These reference sequences exist independently of genome builds. Explain

    These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

    mRNA and Protein(s)

    1. NM_001012177.1 → NP_001012177.1  methylcrotonoyl-CoA carboxylase beta chain, mitochondrial

      See identical proteins and their annotated locations for NP_001012177.1

      Status: PROVISIONAL

      Source sequence(s)
      BC083581
      UniProtKB/Swiss-Prot
      Q5XIT9
      UniProtKB/TrEMBL
      A0A8I5ZSP4
      Related
      ENSRNOP00000023900.5, ENSRNOT00000023900.7
      Conserved Domains (1) summary
      PLN02820
      Location:36 → 563
      PLN02820; 3-methylcrotonyl-CoA carboxylase, beta chain

    RefSeqs of Annotated Genomes: GCF_036323735.1-RS_2024_02

    The following sections contain reference sequences that belong to a specific genome build. Explain

    Reference GRCr8

    Genomic

    1. NC_086020.1 Reference GRCr8

      Range
      33039044..33110092 complement
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

    mRNA and Protein(s)

    1. XM_039102544.2 → XP_038958472.1  methylcrotonoyl-CoA carboxylase beta chain, mitochondrial isoform X1

      UniProtKB/TrEMBL
      A0A8I5ZSP4, A0A8I6A3M2
      Conserved Domains (1) summary
      PLN02820
      Location:36 → 515
      PLN02820; 3-methylcrotonyl-CoA carboxylase, beta chain

    2. XM_039102546.2 → XP_038958474.1  methylcrotonoyl-CoA carboxylase beta chain, mitochondrial isoform X2

      UniProtKB/TrEMBL
      A0A8I5ZSP4
      Conserved Domains (1) summary
      PLN02820
      Location:36 → 496
      PLN02820; 3-methylcrotonyl-CoA carboxylase, beta chain
    Nucleotide Protein Strain
    Heading Accession and Version
    Protein Accession Links
    GenPept Link UniProtKB Link
    Q5XIT9.1 GenPept UniProtKB/Swiss-Prot:Q5XIT9

    Gene LinkOut

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