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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
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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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