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Tbio
MCCC2
Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial

Protein Classes
Protein Summary
Description
Carboxyltransferase subunit of the 3-methylcrotonyl-CoA carboxylase, an enzyme that catalyzes the conversion of 3-methylcrotonyl-CoA to 3-methylglutaconyl-CoA, a critical step for leucine and isovaleric acid catabolism. 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. Alternative splicing results in multiple transcript variants encoding distinct isoforms. [provided by RefSeq, May 2018]
Uniprot Accession IDs
Gene Name
Ensembl ID
  • ENST00000340941
  • ENSP00000343657
  • ENSG00000131844

Symbol
  • MCCB
  • MCCB
Illumination Graph
Knowledge Table
Most Knowledge About
Knowledge Value (0 to 1 scale)
transcription factor perturbation
0.91
molecular function
0.88
histone modification site profile
0.85
transcription factor binding site profile
0.7
transcription factor
0.66


Related Tools
GENEVA
Thumbnail image for GENEVA
GENEVA (GENe Expression Variance Analysis) allows you to identify RNA-sequencing datasets from the Gene Expression Omnibus (GEO) that contain conditions modulating a gene or a gene signature.
GlyGen
Thumbnail image for GlyGen
GlyGen is a data integration and dissemination project for carbohydrate and glycoconjugate related data.
IDG Development Level Summary
Tdark

These are targets about which virtually nothing is known. They do not have known drug or small molecule activities
- AND - satisfy two or more of the following criteria:

Pubmed score: 14.41   (req: < 5)
Gene RIFs: 10   (req: <= 3)
Antibodies: 251   (req: <= 50)
Tbio

These targets do not have known drug or small molecule activities
- AND - satisfy two or more of the following criteria:

Pubmed score: 14.41   (req: >= 5)
Gene RIFs: 10   (req: > 3)
Antibodies: 251   (req: > 50)

- OR - satisfy the following criterion:

Gene Ontology Terms: 6
Tchem

Target has at least one ChEMBL compound with an activity cutoff of < 30 nM - AND - satisfies the preceding conditions

Active Ligand: 0
Tclin

Target has at least one approved drug - AND - satisfies the preceding conditions

Active Drug: 0
Orthologs (9)
1 – 5 of 9
Species
Name
Source ID
Gene ID
OMA
EggNOG
Inparanoid
Mouse
MGI:1925288
78038
Rat
RGD:1310279
361884
Dog
methylcrotonoyl-CoA carboxylase 2
VGNC:43071
478091
Horse
methylcrotonoyl-CoA carboxylase 2
VGNC:20025
100073301
Opossum
methylcrotonoyl-CoA carboxylase 2
103098085
Species
Name
OMA
EggNOG
Inparanoid
Mouse
Rat
Dog
methylcrotonoyl-CoA carboxylase 2
Horse
methylcrotonoyl-CoA carboxylase 2
Opossum
methylcrotonoyl-CoA carboxylase 2
Protein Structure (0 Structures, 1 AlphaFold Model)
RepresentationColor Scheme
AF-Q9HCC0-F1-model_v1

AlphaFold Structures Developed by DeepMind and EMBL-EBI

Pathways (15)
Biotin transport and metabolism (R-HSA-196780)

Click on a row in the table to change the structure displayed.

Items per page:
1 – 5 of 11
Data Source
Name
Explore in Pharos
Explore in Source
Reactome
Biotin transport and metabolism
Reactome
Branched-chain amino acid catabolism
Reactome
Defective HLCS causes multiple carboxylase deficiency
Reactome
Defects in biotin (Btn) metabolism
Reactome
Defects in vitamin and cofactor metabolism
Name
Explore in Pharos
Explore in Source
Biotin transport and metabolism
Branched-chain amino acid catabolism
Defective HLCS causes multiple carboxylase deficiency
Defects in biotin (Btn) metabolism
Defects in vitamin and cofactor metabolism
Gene Ontology Terms (11)
Items per page:
10
1 – 2 of 2
GO Term
Evidence
Assigned by
Inferred from Electronic Annotation (IEA)
UniProtKB-KW
Inferred from Electronic Annotation (IEA)
UniProtKB-EC
Protein-Protein Interactions (86)
1 – 10 of 86
ECH1
Tbio
Family:  Enzyme
Novelty:  0.02471875
p_int:  0.998372367
p_ni:  0.001627633
Score:  0.567
Data Source:  BioPlex,STRINGDB
RECK
Tbio
Novelty:  0.00409224
p_int:  0.998001699
p_ni:  0.001998301
Score:  0.485
Data Source:  BioPlex,STRINGDB
MCCC1
Tbio
Family:  Enzyme
Novelty:  0.01234441
Score:  0.999
Data Source:  Reactome,STRINGDB
IVD
Tbio
Family:  Enzyme
Novelty:  0.03203942
Score:  0.991
Data Source:  STRINGDB
PCCA
Tbio
Family:  Enzyme
Novelty:  0.00515417
Score:  0.988
Data Source:  STRINGDB
ECHS1
Tbio
Family:  Enzyme
Novelty:  0.01291476
Score:  0.977
Data Source:  STRINGDB
AUH
Tbio
Family:  Enzyme
Novelty:  0.01698227
Score:  0.963
Data Source:  STRINGDB
PC
Tbio
Family:  Enzyme
Novelty:  0.00271694
Score:  0.953
Data Source:  STRINGDB
HLCS
Tchem
Family:  Enzyme
Novelty:  0.01597449
Score:  0.947
Data Source:  Reactome,STRINGDB
HADHA
Tbio
Novelty:  0.00385746
Score:  0.944
Data Source:  STRINGDB
Publication Statistics
PubMed Score  14.41

PubMed score by year
PubTator Score  92.86

PubTator score by year
Amino Acid Sequence
Residue Counts
Protein Sequence
ProtVista Viewer