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

Gene ID: 294972, updated on 28-Oct-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, Oct 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

Expression

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  • Project title: A rat RNA-Seq transcriptomic BodyMap across 11 organs and 4 developmental stages 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. 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

  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

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