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Mccc2 methylcrotonyl-CoA carboxylase subunit 2 [ Rattus norvegicus (Norway rat) ]

Gene ID: 361884, updated on 4-Jan-2025

Summary

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Official Symbol
Mccc2provided by RGD
Official Full Name
methylcrotonyl-CoA carboxylase subunit 2provided by RGD
Primary source
RGD:1310279
See related
EnsemblRapid:ENSRNOG00000017752 AllianceGenome:RGD:1310279
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
Contributes to methylcrotonoyl-CoA carboxylase activity. Involved in coenzyme A metabolic 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 2 deficiency and mitochondrial metabolism disease. Orthologous to human MCCC2 (methylcrotonyl-CoA carboxylase subunit 2). [provided by Alliance of Genome Resources, Jan 2025]
Expression
Biased expression in Kidney (RPKM 463.7), Heart (RPKM 405.5) and 9 other tissues See more
Orthologs
human mouse all
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Genomic context

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See Mccc2 in Genome Data Viewer
Location:
2q12
Exon count:
18
Annotation release Status Assembly Chr Location
RS_2024_02 current GRCr8 (GCF_036323735.1) 2 NC_086020.1 (33039044..33110092, complement)
RS_2023_06 previous assembly mRatBN7.2 (GCF_015227675.2) 2 NC_051337.1 (31304927..31375978, complement)
106 previous assembly Rnor_6.0 (GCF_000001895.5) 2 NC_005101.4 (30175017..30246028, complement)

Chromosome 2 - NC_086020.1Genomic Context describing neighboring genes Neighboring gene ubiquitin conjugating enzyme E2 V1, pseudogene 20 Neighboring gene CART prepropeptide Neighboring gene BDP1 general transcription factor IIIB subunit Neighboring gene small EDRK-rich factor 1

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
  • 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. Simultaneous preparation of the three biotin-containing mitochondrial carboxylases from rat liver. Oei J, et al. Biochim Biophys Acta, 1985 May 29. PMID 3995077
  2. Expression, purification, characterization of human 3-methylcrotonyl-CoA carboxylase (MCCC). Chu CH, et al. Protein Expr Purif, 2007 Jun. PMID 17360195
  3. The molecular basis of 3-methylcrotonylglycinuria, a disorder of leucine catabolism. Gallardo ME, et al. Am J Hum Genet, 2001 Feb. PMID 11170888, Free PMC Article
  4. Mitochondrial targeting signals and mature peptides of 3-methylcrotonyl-CoA carboxylase. Stadler SC, et al. Biochem Biophys Res Commun, 2005 Sep 2. PMID 16023992
  5. 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

See all (10) 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

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
  
contributes_to methylcrotonoyl-CoA carboxylase activity IBA
Inferred from Biological aspect of Ancestor
more info
  
contributes_to methylcrotonoyl-CoA carboxylase activity IDA
Inferred from Direct Assay
more info
 PubMed 
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 IBA
Inferred from Biological aspect of Ancestor
more info
  
involved_in L-leucine catabolic process IEA
Inferred from Electronic Annotation
more info
  
involved_in coenzyme A metabolic process IDA
Inferred from Direct Assay
more info
 PubMed 
Component Evidence Code Pubs
part_of methylcrotonoyl-CoA carboxylase complex IBA
Inferred from Biological aspect of Ancestor
more info
  
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 beta chain, mitochondrial
Names
3-methylcrotonyl-CoA carboxylase 2
3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit
3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta
MCCase subunit beta
methylcrotonoyl-CoA carboxylase 2 (beta)
methylcrotonoyl-Coenzyme A carboxylase 2 (beta)
NP_001012177.1
XP_038958472.1
XP_038958474.1

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_001012177.1 → NP_001012177.1  methylcrotonoyl-CoA carboxylase beta chain, mitochondrial

    See identical proteins and their annotated locations for NP_001012177.1

    Status: PROVISIONAL

    Source sequence(s)
    BC083581
    UniProtKB/Swiss-Prot
    Q5XIT9
    UniProtKB/TrEMBL
    A0A8I5ZSP4
    Related
    ENSRNOP00000023900.5, ENSRNOT00000023900.7
    Conserved Domains (1) summary
    PLN02820
    Location:36 → 563
    PLN02820; 3-methylcrotonyl-CoA carboxylase, beta chain

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
    33039044..33110092 complement
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. XM_039102544.2 → XP_038958472.1  methylcrotonoyl-CoA carboxylase beta chain, mitochondrial isoform X1

    UniProtKB/TrEMBL
    A0A8I5ZSP4, A0A8I6A3M2
    Conserved Domains (1) summary
    PLN02820
    Location:36 → 515
    PLN02820; 3-methylcrotonyl-CoA carboxylase, beta chain

  2. XM_039102546.2 → XP_038958474.1  methylcrotonoyl-CoA carboxylase beta chain, mitochondrial isoform X2

    UniProtKB/TrEMBL
    A0A8I5ZSP4
    Conserved Domains (1) summary
    PLN02820
    Location:36 → 496
    PLN02820; 3-methylcrotonyl-CoA carboxylase, beta chain
Nucleotide Protein Strain
Heading Accession and Version
Protein Accession Links
GenPept Link UniProtKB Link
Q5XIT9.1 GenPept UniProtKB/Swiss-Prot:Q5XIT9

Gene LinkOut

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