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    Mccc1 Methylcrotonoyl-CoA carboxylase 1 [ Drosophila melanogaster (fruit fly) ]

    Gene ID: 43750, updated on 9-Dec-2024

    Summary

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    Official Symbol
    Mccc1provided by FlyBase
    Official Full Name
    Methylcrotonoyl-CoA carboxylase 1provided by FlyBase
    Primary source
    FLYBASE:FBgn0039877
    Locus tag
    Dmel_CG2118
    See related
    AllianceGenome:FB:FBgn0039877
    Gene type
    protein coding
    RefSeq status
    REVIEWED
    Organism
    Drosophila melanogaster
    Lineage
    Eukaryota; Metazoa; Ecdysozoa; Arthropoda; Hexapoda; Insecta; Pterygota; Neoptera; Endopterygota; Diptera; Brachycera; Muscomorpha; Ephydroidea; Drosophilidae; Drosophila; Sophophora
    Also known as
    CG2118; Dmel\CG2118; mcc; MCC; Q9V9T5_DROME
    Summary
    Predicted to enable ATP binding activity and metal ion binding activity. Predicted to contribute to methylcrotonoyl-CoA carboxylase activity. Predicted to be located in mitochondrial matrix. Predicted to be part of methylcrotonoyl-CoA carboxylase complex. Predicted to be active in mitochondrion. Is expressed in adult head; adult heart; organism; and spermatozoon. 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]
    Orthologs
    all
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    Genomic context

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    See Mccc1 in Genome Data Viewer
    Location:
    100D2-100D2; 3-102 cM
    Exon count:
    8
    Annotation release Status Assembly Chr Location
    Release 6.54 current Release 6 plus ISO1 MT (GCF_000001215.4) 3R NT_033777.3 (31790592..31793907, complement)
    Release 5.57 previous assembly Release 5 (GCF_000001215.2) 3R NT_033777.2 (27616314..27619629, complement)

    Chromosome 3R - NT_033777.3Genomic Context describing neighboring genes Neighboring gene antisense RNA:CR33945 Neighboring gene beta glucuronidase Neighboring gene septin interacting protein 3 Neighboring gene uncharacterized protein Neighboring gene fat facets Neighboring gene ATP-dependent chromatin assembly factor large subunit Neighboring gene uncharacterized protein Neighboring gene Ionotropic receptor 100a

    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. Biotin controls intestinal stem cell mitosis and host-microbiome interactions. Neophytou C, et al. Cell Rep, 2022 Mar 8. PMID 35263602
    2. Susceptibility to heat stress and aberrant gene expression patterns in holocarboxylase synthetase-deficient Drosophila melanogaster are caused by decreased biotinylation of histones, not of carboxylases. Camporeale G, et al. J Nutr, 2007 Apr. PMID 17374649, Free PMC Article
    3. Functionally Validating Evolutionary Conserved Risk Genes for Parkinson's Disease in Drosophila melanogaster. Baisgaard AE, et al. Insects, 2023 Feb 9. PMID 36835737, Free PMC Article
    4. A Drosophila model of Friedreich ataxia with CRISPR/Cas9 insertion of GAA repeats in the frataxin gene reveals in vivo protection by N-acetyl cysteine. Russi M, et al. Hum Mol Genet, 2020 Oct 10. PMID 32744307
    5. Chromosomal instability causes sensitivity to protein folding stress and ATP depletion. Khan M, et al. Biol Open, 2018 Oct 16. PMID 30327366, Free PMC Article

    See all (51) 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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    Homology

    Gene Ontology Provided by FlyBase

    Function Evidence Code Pubs
    enables ATP binding 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 ISS
    Inferred from Sequence or Structural Similarity
    more info
    		  
    Component Evidence Code Pubs
    part_of methylcrotonoyl-CoA carboxylase complex ISS
    Inferred from Sequence or Structural Similarity
    more info
    		  
    located_in mitochondrial matrix ISS
    Inferred from Sequence or Structural Similarity
    more info
    		  
    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 1
    Names
    3-methyl-crotonyl-CoA carboxylase
    CG2118-PA
    CG2118-PB
    CG2118-PC
    CG2118-PD
    Mccc1-PA
    Mccc1-PB
    Mccc1-PC
    Mccc1-PD

    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. NT_033777.3 Reference assembly

      Range
      31790592..31793907 complement
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

    mRNA and Protein(s)

    1. NM_001260462.2 → NP_001247391.1  Methylcrotonoyl-CoA carboxylase 1, isoform C [Drosophila melanogaster]

      See identical proteins and their annotated locations for NP_001247391.1

      Status: REVIEWED

      UniProtKB/TrEMBL
      Q9V9T5
      Conserved Domains (5) summary
      cd06850
      Location:626 → 691
      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, ...
      smart00878
      Location:362 → 469
      Biotin_carb_C; Biotin carboxylase C-terminal domain
      COG4770
      Location:27 → 693
      PccA; Acetyl/propionyl-CoA carboxylase, alpha subunit [Lipid transport and metabolism]
      pfam00289
      Location:27 → 135
      CPSase_L_chain; Carbamoyl-phosphate synthase L chain, N-terminal domain
      pfam02786
      Location:141 → 348
      CPSase_L_D2; Carbamoyl-phosphate synthase L chain, ATP binding domain

    2. NM_170577.3 → NP_733456.1  Methylcrotonoyl-CoA carboxylase 1, isoform B [Drosophila melanogaster]

      See identical proteins and their annotated locations for NP_733456.1

      Status: REVIEWED

      UniProtKB/TrEMBL
      Q8I0S9
      Related
      FBpp0085214
      Conserved Domains (5) summary
      cd06850
      Location:562 → 627
      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, ...
      smart00878
      Location:298 → 405
      Biotin_carb_C; Biotin carboxylase C-terminal domain
      COG4770
      Location:1 → 629
      PccA; Acetyl/propionyl-CoA carboxylase, alpha subunit [Lipid transport and metabolism]
      pfam00289
      Location:1 → 71
      CPSase_L_chain; Carbamoyl-phosphate synthase L chain, N-terminal domain
      pfam02786
      Location:77 → 284
      CPSase_L_D2; Carbamoyl-phosphate synthase L chain, ATP binding domain

    3. NM_143639.1 → NP_651896.1  Methylcrotonoyl-CoA carboxylase 1, isoform A [Drosophila melanogaster]

      See identical proteins and their annotated locations for NP_651896.1

      Status: REVIEWED

      UniProtKB/TrEMBL
      Q9V9T5
      Related
      FBpp0085215, FBtr0085856
      Conserved Domains (5) summary
      cd06850
      Location:626 → 691
      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, ...
      smart00878
      Location:362 → 469
      Biotin_carb_C; Biotin carboxylase C-terminal domain
      COG4770
      Location:27 → 693
      PccA; Acetyl/propionyl-CoA carboxylase, alpha subunit [Lipid transport and metabolism]
      pfam00289
      Location:27 → 135
      CPSase_L_chain; Carbamoyl-phosphate synthase L chain, N-terminal domain
      pfam02786
      Location:141 → 348
      CPSase_L_D2; Carbamoyl-phosphate synthase L chain, ATP binding domain

    4. NM_001260463.1 → NP_001247392.1  Methylcrotonoyl-CoA carboxylase 1, isoform D [Drosophila melanogaster]

      See identical proteins and their annotated locations for NP_001247392.1

      Status: REVIEWED

      UniProtKB/TrEMBL
      Q8I0S9
      Conserved Domains (5) summary
      cd06850
      Location:562 → 627
      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, ...
      smart00878
      Location:298 → 405
      Biotin_carb_C; Biotin carboxylase C-terminal domain
      COG4770
      Location:1 → 629
      PccA; Acetyl/propionyl-CoA carboxylase, alpha subunit [Lipid transport and metabolism]
      pfam00289
      Location:1 → 71
      CPSase_L_chain; Carbamoyl-phosphate synthase L chain, N-terminal domain
      pfam02786
      Location:77 → 284
      CPSase_L_D2; Carbamoyl-phosphate synthase L chain, ATP binding domain
    Nucleotide Protein
    Heading Accession and Version
    Protein Accession Links
    GenPept Link UniProtKB Link
    Q8I0S9 Q8I0S9
    Q9V9T5 Q9V9T5

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