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

Gene ID: 72039, updated on 1-Mar-2024

Summary

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
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Genomic context

See Mccc1 in Genome Data Viewer
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

Expression

  • 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

Variation

Alleles

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

Pathways from PubChem

Interactions

Products Interactant Other Gene Complex Source Pubs Description

General gene information

Markers

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
 

General protein information

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)

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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_023644.4NP_076133.3  methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial

    See identical proteins and their annotated locations for NP_076133.3

    Status: VALIDATED

    Source sequence(s)
    AA198778, AK031072, AK032203, BY139695
    Consensus CDS
    CCDS17307.1
    UniProtKB/Swiss-Prot
    Q3TGU0, Q99MR8, Q9D8R2
    Related
    ENSMUSP00000029259.4, ENSMUST00000029259.10
    Conserved Domains (5) summary
    cd06850
    Location:647710
    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:45712
    PccA; Acetyl/propionyl-CoA carboxylase, alpha subunit [Lipid transport and metabolism]
    pfam00289
    Location:45153
    CPSase_L_chain; Carbamoyl-phosphate synthase L chain, N-terminal domain
    pfam02785
    Location:379485
    Biotin_carb_C; Biotin carboxylase C-terminal domain
    cl17255
    Location:159365
    ATP-grasp_4; ATP-grasp domain

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