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Tbio
MCCC1
Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial

Protein Classes
Protein Summary
Description
Biotin-attachment 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 large 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]
Uniprot Accession IDs
Gene Name
Ensembl ID
  • ENST00000265594
  • ENSP00000265594
  • ENSG00000078070

Symbol
  • MCCA
  • MCCA
  • MCC-B
Illumination Graph
Knowledge Table
Most Knowledge About
Knowledge Value (0 to 1 scale)
protein domain
1
molecular function
0.99
disease perturbation
0.98
virus perturbation
0.83
histone modification site profile
0.78


Related Tools
Target Illumination GWAS Analytics (TIGA)
TIGA scores and ranks GWAS discovered associations according to the quantity and quality of the evidence supporting the association.
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
GlyGen is a data integration and dissemination project for carbohydrate and glycoconjugate related data.
ARCHS4
ARCHS4 provides access to gene-function predictions based on RNA-seq co-expression, and gene expression levels across cell and tissues.
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: 88.82   (req: < 5)
Gene RIFs: 18   (req: <= 3)
Antibodies: 154   (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: 88.82   (req: >= 5)
Gene RIFs: 18   (req: > 3)
Antibodies: 154   (req: > 50)

- OR - satisfy the following criterion:

Gene Ontology Terms: 8
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
GWAS Traits (4)
GWAS Trait
EFO ID
Study Count
SNP Count
Beta Count
Odds Ratio
Evidence (Mean Rank Score)
Provenance
6
5
1
1.2
98.7
blood metabolite measurement
1
1
1
64.2
lymphocyte count
2
2
2
59
monocyte count
1
1
1
17.5
GWAS Trait
EFO ID
Beta Count
Odds Ratio
Evidence (Mean Rank Score)
Provenance
1
1.2
98.7
blood metabolite measurement
1
64.2
lymphocyte count
2
59
monocyte count
1
17.5
Orthologs (16)
1 – 5 of 16
Species
Name
Source ID
Gene ID
OMA
EggNOG
Inparanoid
Chimp
methylcrotonoyl-CoA carboxylase 1
VGNC:6362
460872
Macaque
methylcrotonoyl-CoA carboxylase 1
708258
Mouse
MGI:1919289
72039
Rat
RGD:1310615
294972
Dog
methylcrotonoyl-CoA carboxylase 1
VGNC:43070
478645
Species
Name
OMA
EggNOG
Inparanoid
Chimp
methylcrotonoyl-CoA carboxylase 1
Macaque
methylcrotonoyl-CoA carboxylase 1
Mouse
Rat
Dog
methylcrotonoyl-CoA carboxylase 1
Protein Structure (1 Structure, 1 AlphaFold Model)
RepresentationColor Scheme

Click on a row in the table to change the structure displayed.
More information can be found at RCSB PDB

AF-Q96RQ3-F1-model_v1

AlphaFold Structures Developed by DeepMind and EMBL-EBI

1 – 1 of 1
PDB Structure Id
Ligand
Method
Resolution (Å)
Residues
Fraction of Total Protein
Pub Year
Title
PDB Structure Id
Fraction of Total Protein
Resolution
Pub Year
Pathways (25)
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 (13)
Items per page:
10
1 – 5 of 5
GO Term
Evidence
Assigned by
Non-traceable Author Statement (NAS)
UniProtKB
Non-traceable Author Statement (NAS)
ParkinsonsUK-UCL
Inferred from Electronic Annotation (IEA)
UniProtKB-KW
Inferred from Electronic Annotation (IEA)
InterPro
Inferred from Electronic Annotation (IEA)
UniProtKB-EC
Protein-Protein Interactions (117)
1 – 10 of 117
ECH1
Tbio
Family: Enzyme
Novelty: 0.02471875
p_int: 0.98732862
p_ni: 0.01267138
Score: 0.262
Data Source: BioPlex,STRINGDB
TUFM
Tbio
Novelty: 0.03251632
p_int: 0.984581967
p_ni: 0.015418033
Score: 0.374
Data Source: BioPlex,STRINGDB
NPB
Tdark
Novelty: 0.00936692
p_int: 0.961365357
p_ni: 0.038634642
p_wrong: 1e-9
Data Source: BioPlex
FOXRED1
Tbio
Family: Enzyme
Novelty: 0.07057772
p_int: 0.953801899
p_ni: 0.046198101
Score: 0.23
Data Source: BioPlex,STRINGDB
TMEM70
Tbio
Novelty: 0.03111438
p_int: 0.91045749
p_ni: 0.08954251
Data Source: BioPlex
NDUFS7
Tclin
Family: Enzyme
Novelty: 0.01562198
p_int: 0.908540831
p_ni: 0.091459138
p_wrong: 3.1e-8
Score: 0.156
Data Source: BioPlex,STRINGDB
SUCLA2
Tbio
Family: Enzyme
Novelty: 0.02029861
p_int: 0.880043022
p_ni: 0.119956963
p_wrong: 1.5e-8
Score: 0.255
Data Source: BioPlex,STRINGDB
GLB1L3
Tdark
Family: Enzyme
Novelty: 1.00833616
p_int: 0.878248889
p_ni: 0.121751111
Data Source: BioPlex
ACSM5
Tbio
Family: Enzyme
Novelty: 0.11562771
p_int: 0.868817517
p_ni: 0.131182483
Score: 0.267
Data Source: BioPlex,STRINGDB
GCAT
Tbio
Family: Enzyme
Novelty: 0.00503538
p_int: 0.853228748
p_ni: 0.146771252
Score: 0.328
Data Source: BioPlex,STRINGDB
Publication Statistics
PubMed Score  88.82

PubMed score by year
PubTator Score  41.82

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