Mccc2 methylcrotonoyl-Coenzyme A carboxylase 2 (beta) [Mus musculus (house mouse)] - Gene

Summary

Official Symbol: Mccc2provided by MGI
Official Full Name: methylcrotonoyl-Coenzyme A carboxylase 2 (beta)provided by MGI
Primary source: MGI:MGI:1925288
See related: Ensembl:ENSMUSG00000021646 AllianceGenome:MGI:1925288
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: MCCB; 4930552N12Rik
Summary: Predicted to enable ATP binding activity. Predicted to contribute to methylcrotonoyl-CoA carboxylase activity. Predicted to be involved in L-leucine catabolic process and coenzyme A metabolic process. Located in mitochondrion. Is expressed in metanephros and spinal cord. 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, Dec 2024]
Expression: Ubiquitous expression in kidney adult (RPKM 24.4), liver adult (RPKM 15.1) and 28 other tissues See more
Orthologs: human all
NEW: Try the new Gene table
Try the new Transcript table

Genomic context

Location:: 13 D1; 13 52.9 cM

Exon count:: 17

Annotation release	Status	Assembly	Chr	Location
RS_2024_02	current	GRCm39 (GCF_000001635.27)	13	NC_000079.7 (100085040..100152147, complement)
108.20200622	previous assembly	GRCm38.p6 (GCF_000001635.26)	13	NC_000079.6 (99948532..100015639, complement)

Chromosome 13 - NC_000079.7 Genomic Context describing neighboring genes

Genomic regions, transcripts, and products

Go to the top of the page Help

Go to reference sequence details

Genomic Sequence:

Go to nucleotide: Graphics FASTA GenBank

Bibliography

Go to the top of the page Help

GeneRIFs: Gene References Into Functions

What's a GeneRIF?

Submit: New GeneRIF Correction

Variation

Go to the top of the page Help

Alleles

Alleles of this type are documented at Mouse Genome Informatics (MGI)

Endonuclease-mediated (2)

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

D13Die20 (e-PCR), detects polymorphism
- UniSTS:141906

D13Die5 (e-PCR), detects polymorphism
- UniSTS:141917

D13Die8 (e-PCR), detects polymorphism
- UniSTS:141920

D13Die38 (e-PCR), detects polymorphism
- UniSTS:186742

D13Die39 (e-PCR), detects polymorphism
- UniSTS:186743

D13Die40 (e-PCR), detects polymorphism
- UniSTS:186744

11.MMHAP69FLD2.seq (e-PCR)
- UniSTS:123087

Gene Ontology Provided by MGI

Function	Evidence Code	Pubs
enables ATP 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	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	IEA Inferred from Electronic Annotation more info
involved_in coenzyme A metabolic process	ISO Inferred from Sequence Orthology more info

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	IEA Inferred from Electronic Annotation more info
located_in mitochondrial matrix	ISO Inferred from Sequence Orthology more info
located_in mitochondrion	HDA more info	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 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

NP_084302.1

EC 6.4.1.4

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)

NM_030026.2 → NP_084302.1 methylcrotonoyl-CoA carboxylase beta chain, mitochondrial

See identical proteins and their annotated locations for NP_084302.1

Status: VALIDATED

Source sequence(s)

AK145564, BC058987, BY237319

Consensus CDS

CCDS36764.1

UniProtKB/Swiss-Prot

Q3ULD5, Q3UPS6

UniProtKB/TrEMBL

B2RUK5

Related

ENSMUSP00000022148.7, ENSMUST00000022148.7

Conserved Domains (2) summary

PLN02820
Location:8 → 563

PLN02820; 3-methylcrotonyl-CoA carboxylase, beta chain

cl23717
Location:84 → 255

crotonase-like; Crotonase/Enoyl-Coenzyme A (CoA) hydratase superfamily. This superfamily contains a diverse set of enzymes including enoyl-CoA hydratase, napthoate synthase, methylmalonyl-CoA decarboxylase, 3-hydoxybutyryl-CoA dehydratase, and dienoyl-CoA isomerase. ...