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MCCC2

GENERAL INFORMATION

Gene name

 MCCC2

Gene description

 Methylcrotonoyl-CoA carboxylase 2 (beta)

Protein class

 Disease related genes
Enzymes
Potential drug targets
Predicted intracellular proteins

Predicted localization

 Intracellular

Number of transcripts

 4
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HUMAN PROTEIN ATLAS INFORMATION

RNA tissue category
HPA:Expressed in all
GTEx:Expressed in all
FANTOM5:Expressed in all

Protein evidence

 Evidence at protein level

Protein expression

 Cytoplasmic expression in most tissues.

DATA RELIABILITY

Data reliability
description

 Antibody staining mainly consistent with RNA expression data. Pending external verification.

Reliability score

 Supported based on 3 antibodies.
HPA038300 , HPA038301 , HPA061546
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RNA AND PROTEIN EXPRESSION SUMMARY
RNA expression (TPM) Protein expression (score)
Brain
Caudate
N/A
Endocrine tissues
Bone marrow & immune system
Muscle tissues
Lung
Bronchus
N/A
Liver & gallbladder
Pancreas
Gastrointestinal tract
Kidney & urinary bladder
Male tissues
Female tissues
Vagina
N/A
Adipose & soft tissue
Skin
Cerebral cortex

Lymph node

Liver

Colon

Kidney

Testis

Skin

PROTEIN EXPRESSION OVERVIEW
Organ
Expression
Alphabetical

RNA EXPRESSION OVERVIEW
HPA dataset
Organ
Expression
Alphabetical
RNA tissue category: Expressed in all
GTEx dataset
Organ
Expression
Alphabetical
RNA tissue category: Expressed in all
FANTOM5 dataset
Organ
Expression
Alphabetical
RNA tissue category: Expressed in all

GENE INFORMATION

Gene name

 MCCC2 (HGNC Symbol)

Synonyms

 MCCB

Description

 Methylcrotonoyl-CoA carboxylase 2 (beta) (HGNC Symbol)

Entrez gene summary

 This gene encodes the small subunit of 3-methylcrotonyl-CoA carboxylase. This enzyme functions as a heterodimer and catalyzes the carboxylation of 3-methylcrotonyl-CoA to form 3-methylglutaconyl-CoA. Mutations in this gene are associated with 3-Methylcrotonylglycinuria, an autosomal recessive disorder of leucine catabolism. [provided by RefSeq, Jul 2008]

Chromosome

 5

Cytoband

 q13.2

Chromosome location (bp)

 71587288 - 71658704

Protein evidence

 Evidence at protein level (all genes)

Ensembl

 ENSG00000131844 (version 83.38)

Entrez gene

 64087

UniProt

 Q9HCC0 (UniProt - Evidence at protein level)

neXtProt

 NX_Q9HCC0

Antibodypedia

 MCCC2 antibodies

PROTEIN BROWSER
MCCC2-001
MCCC2-003
MCCC2-009
MCCC2-201

PROTEIN INFORMATION

Splice variant

UniProt

Protein class

Gene ontology

Length & mass

Signal peptide
(predicted)

Transmembrane regions
(predicted)
MCCC2-001
ENSP00000343657
ENST00000340941
Q9HCC0 [Direct mapping]
Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial
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Enzymes
   ENZYME proteins
   Ligase
Predicted intracellular proteins
Disease related genes
Potential drug targets
Protein evidence (Kim et al 2014)
Protein evidence (Ezkurdia et al 2014)
Show all
GO:0004485 [methylcrotonoyl-CoA carboxylase activity]
GO:0005515 [protein binding]
GO:0005524 [ATP binding]
GO:0005739 [mitochondrion]
GO:0005759 [mitochondrial matrix]
GO:0005829 [cytosol]
GO:0006552 [leucine catabolic process]
GO:0006766 [vitamin metabolic process]
GO:0006767 [water-soluble vitamin metabolic process]
GO:0006768 [biotin metabolic process]
GO:0009083 [branched-chain amino acid catabolic process]
GO:0015936 [coenzyme A metabolic process]
GO:0016874 [ligase activity]
GO:0034641 [cellular nitrogen compound metabolic process]
GO:0044281 [small molecule metabolic process]
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 563 aa
61.3 kDa 		No 0
MCCC2-003
ENSP00000420994
ENST00000509358
D6RDF7 [Direct mapping]
Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial
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Predicted intracellular proteins
Protein evidence (Ezkurdia et al 2014)
Show all
GO:0008152 [metabolic process]
GO:0016874 [ligase activity]
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 363 aa
39.7 kDa 		No 0
MCCC2-009
ENSP00000425474
ENST00000509539
D6RD67 [Direct mapping]
Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial
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Predicted intracellular proteins
Protein evidence (Ezkurdia et al 2014)
Show all
GO:0005739 [mitochondrion]
GO:0008152 [metabolic process]
GO:0016874 [ligase activity]
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 286 aa
31.6 kDa 		No 0
MCCC2-201
ENSP00000486535
ENST00000629193
D6R9R1 [Direct mapping]
Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial; Methylcrotonoyl-Coenzyme A carboxylase 2 (Beta), isoform CRA_a
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Predicted intracellular proteins
Protein evidence (Ezkurdia et al 2014)
Show all
GO:0008152 [metabolic process]
GO:0016874 [ligase activity]
Show all
 329 aa
36.3 kDa 		No 0

Contact

The Project

The Human Protein Atlas

The Human Protein Atlas project is funded
by the Knut & Alice Wallenberg foundation.