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    Mccc1 methylcrotonyl-CoA carboxylase subunit 1 [ Rattus norvegicus (Norway rat) ]

    Gene ID: 294972, updated on 9-Dec-2024

    Summary

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    Official Symbol
    Mccc1provided by RGD
    Official Full Name
    methylcrotonyl-CoA carboxylase subunit 1provided by RGD
    Primary source
    RGD:1310615
    See related
    EnsemblRapid:ENSRNOG00000013293 AllianceGenome:RGD:1310615
    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
    Predicted to enable ATP binding activity; ligase activity; and metal ion binding activity. Predicted to contribute to methylcrotonoyl-CoA carboxylase activity. Predicted to be involved in L-leucine catabolic 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 1 deficiency. Orthologous to human MCCC1 (methylcrotonyl-CoA carboxylase subunit 1). [provided by Alliance of Genome Resources, Dec 2024]
    Expression
    Biased expression in Kidney (RPKM 356.2), Heart (RPKM 233.0) and 9 other tissues See more
    Orthologs
    human mouse all
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    Genomic context

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    See Mccc1 in Genome Data Viewer
    Location:
    2q25
    Exon count:
    22
    Annotation release Status Assembly Chr Location
    RS_2024_02 current GRCr8 (GCF_036323735.1) 2 NC_086020.1 (120727313..120779334, complement)
    RS_2023_06 previous assembly mRatBN7.2 (GCF_015227675.2) 2 NC_051337.1 (118799147..118851181, complement)
    106 previous assembly Rnor_6.0 (GCF_000001895.5) 2 NC_005101.4 (122550777..122690540, complement)

    Chromosome 2 - NC_086020.1Genomic Context describing neighboring genes Neighboring gene ATPase phospholipid transporting 11B (putative) Neighboring gene uncharacterized LOC134485899 Neighboring gene defective in cullin neddylation 1 domain containing 1 Neighboring gene coiled-coil domain containing 144B Neighboring gene small ribosomal subunit protein uS13-like Neighboring gene uncharacterized LOC134485901

    Genomic regions, transcripts, and products

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    Go to nucleotide: Graphics FASTA GenBank

    Bibliography

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    Related articles in PubMed

    1. Expression, purification, characterization of human 3-methylcrotonyl-CoA carboxylase (MCCC). Chu CH, et al. Protein Expr Purif, 2007 Jun. PMID 17360195
    2. Mitochondrial targeting signals and mature peptides of 3-methylcrotonyl-CoA carboxylase. Stadler SC, et al. Biochem Biophys Res Commun, 2005 Sep 2. PMID 16023992
    3. 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
    4. Proteomic analysis of the mouse liver mitochondrial inner membrane. Da Cruz S, et al. J Biol Chem, 2003 Oct 17. PMID 12865426
    5. Integrated analysis of protein composition, tissue diversity, and gene regulation in mouse mitochondria. Mootha VK, et al. Cell, 2003 Nov 26. PMID 14651853

    See all (8) 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

    Clone Names

    • MGC95141

    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
    		  
    enables metal ion binding 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 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 IEA
    Inferred from Electronic Annotation
    more info
    		  
    involved_in carboxylic acid metabolic process IEA
    Inferred from Electronic Annotation
    more info
    		  
    Component Evidence Code Pubs
    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 subunit alpha, mitochondrial
    Names
    3-methylcrotonyl-CoA carboxylase 1
    3-methylcrotonyl-CoA carboxylase biotin-containing subunit
    3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha
    MCCase subunit alpha
    methylcrotonoyl-CoA carboxylase 1 (alpha)
    methylcrotonoyl-Coenzyme A carboxylase 1 (alpha)
    NP_001009653.1
    XP_038957916.1
    XP_038957917.1
    XP_063137634.1

    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_001009653.1 → NP_001009653.1  methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial

      See identical proteins and their annotated locations for NP_001009653.1

      Status: PROVISIONAL

      Source sequence(s)
      BC088473
      UniProtKB/Swiss-Prot
      Q5I0C3
      UniProtKB/TrEMBL
      F1LP30
      Related
      ENSRNOP00000018942.4, ENSRNOT00000018942.6
      Conserved Domains (5) summary
      cd06850
      Location:647 → 710
      biotinyl_domain; 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, ...
      COG4770
      Location:45 → 712
      PccA; Acetyl/propionyl-CoA carboxylase, alpha subunit [Lipid transport and metabolism]
      pfam00289
      Location:45 → 153
      CPSase_L_chain; Carbamoyl-phosphate synthase L chain, N-terminal domain
      pfam02785
      Location:379 → 485
      Biotin_carb_C; Biotin carboxylase C-terminal domain
      pfam02786
      Location:159 → 365
      CPSase_L_D2; Carbamoyl-phosphate synthase L chain, ATP binding domain

    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
      120727313..120779334 complement
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

    mRNA and Protein(s)

    1. XM_039101988.2 → XP_038957916.1  methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform X1

      UniProtKB/TrEMBL
      A0A8I6AS47, F1LP30
      Related
      ENSRNOP00000094692.1, ENSRNOT00000105123.2
      Conserved Domains (1) summary
      cl39082
      Location:45 → 614
      PycA; Pyruvate carboxylase [Energy production and conversion]

    2. XM_063281564.1 → XP_063137634.1  methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform X2

      UniProtKB/TrEMBL
      A0A8I6AQ31

    3. XM_039101989.2 → XP_038957917.1  methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform X3

      Conserved Domains (1) summary
      cl39082
      Location:45 → 315
      PycA; Pyruvate carboxylase [Energy production and conversion]

    RNA

    1. XR_005500259.2 RNA Sequence

      Related
      ENSRNOT00000132682.1
    Nucleotide Protein Strain
    Heading Accession and Version
    Protein Accession Links
    GenPept Link UniProtKB Link
    Q5I0C3.1 GenPept UniProtKB/Swiss-Prot:Q5I0C3

    Gene LinkOut

    The following LinkOut resources are supplied by external providers. These providers are responsible for maintaining the links.

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