U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Mccc1 methylcrotonoyl-Coenzyme A carboxylase 1 (alpha) [ Mus musculus (house mouse) ]

Gene ID: 72039, updated on 13-Apr-2024

Summary

Go to the top of the page Help
Official Symbol
Mccc1provided by MGI
Official Full Name
methylcrotonoyl-Coenzyme A carboxylase 1 (alpha)provided by MGI
Primary source
MGI:MGI:1919289
See related
Ensembl:ENSMUSG00000027709 AllianceGenome:MGI:1919289
Gene type
protein coding
RefSeq status
VALIDATED
Organism
Mus musculus
Lineage
Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae; Murinae; Mus; Mus
Also known as
Mcca; MCCalpha; 1810045E08Rik; 2310058B18Rik
Summary
Predicted to enable ATP binding activity; ligase activity; and metal ion binding activity. Predicted to contribute to methylcrotonoyl-CoA carboxylase activity. Located in mitochondrial inner membrane. Is expressed in several structures, including alimentary system; genitourinary system; heart; nervous system; and sensory organ. 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, Apr 2022]
Expression
Ubiquitous expression in kidney adult (RPKM 18.2), subcutaneous fat pad adult (RPKM 12.9) and 28 other tissues See more
Orthologs
human all
NEW
Try the new Gene table
Try the new Transcript table

Genomic context

Go to the top of the page Help
Location:
3 B; 3 17.41 cM
Exon count:
20
Annotation release Status Assembly Chr Location
RS_2024_02 current GRCm39 (GCF_000001635.27) 3 NC_000069.7 (36009584..36054843, complement)
108.20200622 previous assembly GRCm38.p6 (GCF_000001635.26) 3 NC_000069.6 (35956292..36000694, complement)

Chromosome 3 - NC_000069.7Genomic Context describing neighboring genes Neighboring gene chaperonin containing Tcp1, subunit 8 (theta) pseudogene Neighboring gene defective in cullin neddylation 1 domain containing 1 Neighboring gene predicted gene 15952 Neighboring gene STARR-positive B cell enhancer ABC_E650 Neighboring gene methylcrotonoyl-Coenzyme A carboxylase 1 (alpha), opposite strand Neighboring gene gene model 57858 Neighboring gene STARR-positive B cell enhancer ABC_E3429 Neighboring gene acyl-Coenzyme A dehydrogenase family, member 9

Genomic regions, transcripts, and products

Go to the top of the page Help

Go to nucleotide: Graphics FASTA GenBank

Expression

Go to the top of the page Help
  • Project title: Mouse ENCODE transcriptome data
  • Description: RNA profiling data sets generated by the Mouse ENCODE project.
  • BioProject: PRJNA66167
  • Publication: PMID 25409824
  • Analysis date: n/a

Bibliography

Go to the top of the pageHelp

Related articles in PubMed

  1. Human biotin-containing subunit of 3-methylcrotonyl-CoA carboxylase gene (MCCA): cDNA sequence, genomic organization, localization to chromosomal band 3q27, and expression. Obata K, et al. Genomics, 2001 Mar 1. PMID 11401427
  2. The molecular basis of human 3-methylcrotonyl-CoA carboxylase deficiency. Baumgartner MR, et al. J Clin Invest, 2001 Feb. PMID 11181649, Free PMC Article
  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. Short-chain 3-hydroxyacyl-coenzyme A dehydrogenase associates with a protein super-complex integrating multiple metabolic pathways. Narayan SB, et al. PLoS One, 2012. PMID 22496890, Free PMC Article
  5. Characterization of the Cardiac Overexpression of HSPB2 Reveals Mitochondrial and Myogenic Roles Supported by a Cardiac HspB2 Interactome. Grose JH, et al. PLoS One, 2015. PMID 26465331, Free PMC Article

See all (33) citations in PubMed

GeneRIFs: Gene References Into Functions

Variation

Go to the top of the page Help

Alleles

Alleles of this type are documented at Mouse Genome Informatics  (MGI)
  • Endonuclease-mediated (1) 

Pathways from PubChem

Go to the top of the page

Interactions

Go to the top of the page Help
Products Interactant Other Gene Complex Source Pubs Description

General gene information

Go to the top of the page Help

Markers

Homology

Gene Ontology Provided by MGI

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
  
enables nucleotide binding IEA
Inferred from Electronic Annotation
more info
  
Component Evidence Code Pubs
part_of 3-methylcrotonyl-CoA carboxylase complex, mitochondrial ISO
Inferred from Sequence Orthology
more info
  
part_of methylcrotonoyl-CoA carboxylase complex ISO
Inferred from Sequence Orthology
more info
  
located_in mitochondrial inner membrane HDA PubMed 
located_in mitochondrial matrix ISO
Inferred from Sequence Orthology
more info
  
located_in mitochondrion HDA PubMed 
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

Go to the top of the page Help
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
NP_076133.3

NCBI Reference Sequences (RefSeq)

Go to the top of the page Help

NEW Try the new Transcript table

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_023644.5 → NP_076133.3  methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial

    See identical proteins and their annotated locations for NP_076133.3

    Status: VALIDATED

    Source sequence(s)
    AC116677
    Consensus CDS
    CCDS17307.1
    UniProtKB/Swiss-Prot
    Q3TGU0, Q99MR8, Q9D8R2
    Related
    ENSMUSP00000029259.4, ENSMUST00000029259.10
    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
    cl17255
    Location:159 → 365
    ATP-grasp_4; ATP-grasp domain

RNA

  1. NR_190638.1 RNA Sequence

    Status: VALIDATED

    Source sequence(s)
    AC116677

  2. NR_190639.1 RNA Sequence

    Status: VALIDATED

    Source sequence(s)
    AC116677

RefSeqs of Annotated Genomes: GCF_000001635.27-RS_2024_02

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

Reference GRCm39 C57BL/6J

Genomic

  1. NC_000069.7 Reference GRCm39 C57BL/6J

    Range
    36009584..36054843 complement
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

RNA

  1. XR_001783756.3 RNA Sequence

  2. XR_001783757.2 RNA Sequence

Nucleotide Protein Strain
Heading Accession and Version
Protein Accession Links
GenPept Link UniProtKB Link
Q99MR8.2 GenPept UniProtKB/Swiss-Prot:Q99MR8

Gene LinkOut

The following LinkOut resources are supplied by external providers. These providers are responsible for maintaining the links.

Chemical Information
Molecular Biology Databases
Research Materials
Tools
Miscellaneous