Mccc2 MGI Mouse Gene Detail - MGI:1925288 - methylcrotonoyl-Coenzyme A carboxylase 2 (beta)

Symbol

Mccc2
Name

methylcrotonoyl-Coenzyme A carboxylase 2 (beta)
Synonyms

4930552N12Rik

Feature Type

protein coding gene
IDs

MGI:1925288
NCBI Gene: 78038
Alliance

gene page
Transcription Start Sites

3 TSS

Sequence Map

Chr13:100085040-100152147 bp, - strand
From NCBI annotation of GRCm39
View this region in JBrowse
Genome Browsers

Ensembl | UCSC | NCBI

Genetic Map

Chromosome 13, 52.90 cM
Mapping Data

1 experiment

SNPs within 2kb

2440 from dbSNP Build 142
Strain Annotations

18

For selected strains:

Strain	Gene Model ID	Feature Type	Coordinates
C57BL/6J	MGI_C57BL6J_1925288	protein coding gene	Chr13:100085038-100152147 (-)
129S1/SvImJ	MGP_129S1SvImJ_G0020878	protein coding gene	Chr13:101765394-101840683 (-)
A/J	MGP_AJ_G0020832	protein coding gene	Chr13:97845924-97913674 (-)
AKR/J	MGP_AKRJ_G0020806	protein coding gene	Chr13:100844257-100914988 (-)
BALB/cJ	MGP_BALBcJ_G0020830	protein coding gene	Chr13:98553876-98630475 (-)
C3H/HeJ	MGP_C3HHeJ_G0020621	protein coding gene	Chr13:100560751-100637774 (-)
C57BL/6NJ	MGP_C57BL6NJ_G0021264	protein coding gene	Chr13:104774314-104865837 (-)
CAROLI/EiJ	MGP_CAROLIEiJ_G0018833	protein coding gene	Chr13:93572478-93636924 (-)
CAST/EiJ	MGP_CASTEiJ_G0020139	protein coding gene	Chr13:100005784-100079280 (-)
CBA/J	MGP_CBAJ_G0020581	protein coding gene	Chr13:108960769-109042318 (-)
DBA/2J	MGP_DBA2J_G0020706	protein coding gene	Chr13:97618542-97688045 (-)
FVB/NJ	MGP_FVBNJ_G0020687	protein coding gene	Chr13:96363126-96432810 (-)
LP/J	MGP_LPJ_G0020781	protein coding gene	Chr13:101857119-101930392 (-)
NOD/ShiLtJ	MGP_NODShiLtJ_G0020715	protein coding gene	Chr13:109213373-109289560 (-)
NZO/HlLtJ	MGP_NZOHlLtJ_G0021305	protein coding gene	Chr13:100294674-100374630 (-)
PWK/PhJ	MGP_PWKPhJ_G0019892	protein coding gene	Chr13:96448687-96517555 (-)
SPRET/EiJ	MGP_SPRETEiJ_G0019715	protein coding gene	Chr13:96577584-96658190 (-)
WSB/EiJ	MGP_WSBEiJ_G0020197	protein coding gene	Chr13:100731878-100802846 (-)

Human Ortholog

MCCC2, methylcrotonyl-CoA carboxylase subunit 2

Vertebrate Orthologs

3

Vertebrate Orthology Source

Alliance of Genome Resources

Human Ortholog

MCCC2, methylcrotonyl-CoA carboxylase subunit 2
Synonyms

MCCB, MCCCbeta
Links

HGNC:6937

NCBI Gene ID: 64087

neXtProt AC: NX_Q9HCC0

UniProt: Q9HCC0
Chr Location

5q13.2; chr5:71579531-71658706 (+) GRCh38

MGI Vertebrate Homology

Mccc2 stringent orthology
1 human;1 mouse;1 rat;1 zebrafish
HCOP

vertebrate homology predictions: MCCC2
Gene Tree

Mccc2

Diseases

2 with human MCCC2 associations

				Human Disease	Mouse Models
				3-methylcrotonyl-CoA carboxylase 2 deficiency IDs 3-methylcrotonyl-CoA carboxylase 2 deficiency DOID:0080580 MESH:C535309 OMIM:210210
				mitochondrial metabolism disease IDs mitochondrial metabolism disease DOID:700 MESH:D028361 UMLS_CUI:C0751651

Click on a disease name to see all genes associated with that disease.

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Phenotype Summary

6 phenotypes from 1 allele in 1 genetic background
8 phenotype references

Phenotype Overview

Blue squares indicate phenotypes directly attributed to mutations/alleles of this gene.

adipose tissue	behavior/neurological	cardiovascular system	cellular	craniofacial	digestive/alimentary system	embryo	endocrine/exocrine glands	growth/size/body	hearing/vestibular/ear	hematopoietic system	homeostasis/metabolism	integument	immune system	limbs/digits/tail	liver/biliary system	mortality/aging	muscle	nervous system	pigmentation	renal/urinary system	reproductive system	respiratory system	skeleton	taste/olfaction	neoplasm	vision/eye

Click cells to view annotations.

All Mutations and Alleles

6
Endonuclease-mediated

2
Gene trapped

3
Targeted

1
Incidental Mutations

Mutagenetix , APF
Find Mice (IMSR)

24 strains or lines available
Comparison Matrix

Gene Expression + Phenotype

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All GO Annotations

17
GO References

10

Molecular Function

carbohydrate derivative binding	cytoskeletal protein binding	DNA binding	enzyme regulator	hydrolase	ligase	lipid binding	oxidoreductase	RNA binding	signaling receptor activity	signaling receptor binding	transcription	transferase	transporter

Biological Process

carbohydrate derivative metabolism	cell differentiation	cell population proliferation	cellular component organization	DNA-templated transcription	establishment of localization	homeostatic process	immune system process	lipid metabolic process	programmed cell death	protein metabolic process	response to stimulus	signaling	system development

Cellular Component

cell projection	cytoplasmic vesicle	cytoskeleton	cytosol	endoplasmic reticulum	endosome	extracellular region	Golgi apparatus	mitochondrion	membraneless organelle	nucleus	organelle envelope	organelle lumen	plasma membrane	protein-containing complex	synapse	vacuole

Click cells to view annotations.

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Expression Overview

GXD's primary emphasis is on endogenous gene expression during development. Click on grid cells to view annotations.

Blue cells = expressed in wild-type.
Gray triangles = other expression annotations only
(e.g. absence of expression or data from mutants).

early conceptus	embryo ectoderm	embryo endoderm	embryo mesoderm	embryo mesenchyme	extraembryonic component	alimentary system	auditory system	branchial arches	cardiovascular system	connective tissue	endocrine system	exocrine system	hemolymphoid system	integumental system	limbs	liver and biliary system	musculoskeletal system	nervous system	olfactory system	reproductive system	respiratory system	urinary system	visual system

Click cells to view annotations.

Assay Results

813
Tissues

98
cDNA Data

62
Literature Summary

2

Images

Tissue x Stage Matrix

Comparison Matrix

Gene Expression + Phenotype

Other Mouse Links

Allen Institute

GEO

Expression Atlas
Other Vertebrate Links

Xenbase mccc2

ZFIN mccc2

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All Sequences

59
RefSeq

2
UniProt

6
CCDS

CCDS36764.1

Ensembl

ENSMUSG00000021646 (Evidence)
NCBI Gene

78038

			Length	Strain/Species	Flank
genomic	78038	NCBI Gene Model \| MGI Sequence Detail	67108	C57BL/6J	± kb
transcript	NM_030026	RefSeq \| MGI Sequence Detail	2146	C57BL/6
polypeptide	Q3ULD5	UniProt \| EBI \| MGI Sequence Detail	563	Not Applicable

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UniProt

6 Sequences
Protein Ontology

PR:000010234 methylcrotonoyl-CoA carboxylase beta chain, mitochondrial

(term hierarchy)
EC

6.4.1.4
InterPro Domains

IPR034733 Acetyl-coenzyme A carboxylase carboxyl transferase subunit beta

IPR011763 Acetyl-coenzyme A carboxyltransferase, C-terminal

IPR011762 Acetyl-coenzyme A carboxyltransferase, N-terminal

IPR029045 ClpP/crotonase-like domain superfamily

IPR045190 Methylcrotonoyl-CoA carboxylase beta chain MCCB/AccD1-like
GlyGen

Q3ULD5 1 site, 1 O-linked glycan (1 site)

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All nucleic 65

cDNA 62

Primer pair 3

Microarray probesets 5

Summaries

All 39

Developmental Gene Expression 2

Gene Ontology 10

Phenotypes 8
Earliest

J:65060 Kawai J, et al., Functional annotation of a full-length mouse cDNA collection. Nature. 2001 Feb 8;409(6821):685-90
Latest

J:345621 Adams DJ, et al., Genetic determinants of micronucleus formation in vivo. Nature. 2024 Mar;627(8002):130-136

TSS for Mccc2:

(View these features in JBrowse)

Transcription Start Site	Location	Distance from Gene 5'-end
Tssr123766	Chr13:100152070-100152155 (-)	34 bp
Tssr123765	Chr13:100152040-100152051 (-)	101 bp
Tssr123764	Chr13:100149006-100149009 (-)	3,139 bp

Contributing Projects: Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
Citing These Resources Funding Information Warranty Disclaimer, Privacy Notice, Licensing, & Copyright Send questions and comments to User Support.	last database update 12/10/2024 MGI 6.24