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    mccc-1 Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial [ Caenorhabditis elegans ]

    Gene ID: 177841, updated on 9-Dec-2024

    Summary

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    Official Symbol
    mccc-1
    Official Full Name
    Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial
    Primary source
    WormBase:WBGene00009319
    Locus tag
    CELE_F32B6.2
    See related
    AllianceGenome:WB:WBGene00009319
    Gene type
    protein coding
    RefSeq status
    REVIEWED
    Organism
    Caenorhabditis elegans (strain: Bristol N2)
    Lineage
    Eukaryota; Metazoa; Ecdysozoa; Nematoda; Chromadorea; Rhabditida; Rhabditina; Rhabditomorpha; Rhabditoidea; Rhabditidae; Peloderinae; Caenorhabditis
    Summary
    Predicted to enable ATP binding activity and ligase activity. Predicted to contribute to methylcrotonoyl-CoA carboxylase activity. Predicted to be active in mitochondrion. Is expressed in several structures, including anal depressor muscle; egg-laying apparatus; intestine; nervous system; and rectal gland cell. 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]
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    Genomic context

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    See mccc-1 in Genome Data Viewer
    Location:
    chromosome: IV
    Exon count:
    9
    Sequence:
    Chromosome: IV; NC_003282.8 (9884570..9887151)

    Chromosome IV - NC_003282.8Genomic Context describing neighboring genes Neighboring gene ncRNA Neighboring gene Nuclear hormone receptor family member nhr-4 Neighboring gene Pre-mRNA-splicing factor 18 Neighboring gene ncRNA Neighboring gene non-specific serine/threonine protein kinase Neighboring gene SH2 domain-containing protein Neighboring gene Sperm-Specific family, class S

    Genomic regions, transcripts, and products

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    Genomic Sequence:
    NC_003282.8

    Go to nucleotide: Graphics FASTA GenBank

    Bibliography

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    Related articles in PubMed

    1. Mitochondrial SIRT4-type proteins in Caenorhabditis elegans and mammals interact with pyruvate carboxylase and other acetylated biotin-dependent carboxylases. Wirth M, et al. Mitochondrion, 2013 Nov. PMID 23438705, Free PMC Article
    2. Integration of metabolic and gene regulatory networks modulates the C. elegans dietary response. Watson E, et al. Cell, 2013 Mar 28. PMID 23540702, Free PMC Article
    3. Pathogenesis of human mitochondrial diseases is modulated by reduced activity of the ubiquitin/proteasome system. Segref A, et al. Cell Metab, 2014 Apr 1. PMID 24703696
    4. SKIP and BIR-1/Survivin have potential to integrate proteome status with gene expression. Kostrouch D, et al. J Proteomics, 2014 Oct 14. PMID 25088050
    5. Gene regulatory network inference in long-lived C. elegans reveals modular properties that are predictive of novel aging genes. Suriyalaksh M, et al. iScience, 2022 Jan 21. PMID 35036864, Free PMC Article

    See all (17) citations in PubMed

    GeneRIFs: Gene References Into Functions

    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 WormBase

    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
    		  
    Component Evidence Code Pubs
    is_active_in mitochondrion IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  

    General protein information

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    Preferred Names
    Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial
    NP_501777.2
    • Confirmed by transcript evidence

    NCBI Reference Sequences (RefSeq)

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    Genome Annotation

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

    Reference assembly

    Genomic

    1. NC_003282.8 Reference assembly

      Range
      9884570..9887151
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

    mRNA and Protein(s)

    1. NM_069376.8 → NP_501777.2  Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial [Caenorhabditis elegans]

      See identical proteins and their annotated locations for NP_501777.2

      Status: REVIEWED

      UniProtKB/TrEMBL
      O45430
      Conserved Domains (5) summary
      cd06850
      Location:606 → 670
      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:19 → 671
      PccA; Acetyl/propionyl-CoA carboxylase, alpha subunit [Lipid transport and metabolism]
      pfam00289
      Location:19 → 127
      CPSase_L_chain; Carbamoyl-phosphate synthase L chain, N-terminal domain
      pfam02785
      Location:353 → 457
      Biotin_carb_C; Biotin carboxylase C-terminal domain
      cl17255
      Location:133 → 339
      ATP-grasp_4; ATP-grasp domain
    Nucleotide Protein
    Heading Accession and Version

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