Gene
mccc1
- ID
- ZDB-GENE-050208-450
- Name
- methylcrotonyl-CoA carboxylase subunit
- Symbol
- mccc1 Nomenclature History
- Previous Names
-
- si:dkey-37m8.6
- zgc:154106
- Type
- protein_coding_gene
- Location
- Chr: 22 Mapping Details/Browsers
- Description
- Predicted to have ATP binding activity; ligase activity; and metal ion binding activity. Human ortholog(s) of this gene implicated in 3-Methylcrotonyl-CoA carboxylase 1 deficiency. Is expressed in liver. Orthologous to human MCCC1 (methylcrotonoyl-CoA carboxylase 1).
- Genome Resources
- Note
- None
- Comparative Information
-
- All Expression Data
- 1 figure from Yossa et al., 2011
- Cross-Species Comparison
- High Throughput Data
- Thisse Expression Data
- No data available
Wild Type Expression Summary
- All Phenotype Data
- No data available
- Cross-Species Comparison
- Alliance
Phenotype Summary
Mutations
Human Disease
| Disease Ontology Term | Multi-Species Data | OMIM Term | OMIM Phenotype ID |
|---|---|---|---|
| 3-methylcrotonyl-CoA carboxylase 1 deficiency | Alliance | 3-Methylcrotonyl-CoA carboxylase 1 deficiency | 210200 |
Domain, Family, and Site Summary
| Type | InterPro ID | Name |
|---|---|---|
| Binding_site | IPR001882 | Biotin-binding site |
| Domain | IPR000089 | Biotin/lipoyl attachment |
| Domain | IPR005479 | Carbamoyl-phosphate synthetase large subunit-like, ATP-binding domain |
| Domain | IPR005481 | Biotin carboxylase-like, N-terminal domain |
| Domain | IPR005482 | Biotin carboxylase, C-terminal |
| Domain | IPR011761 | ATP-grasp fold |
| Domain | IPR011764 | Biotin carboxylation domain |
| Family | IPR050856 | Biotin-dependent Carboxylase Complex |
| Homologous_superfamily | IPR011053 | Single hybrid motif |
| Homologous_superfamily | IPR011054 | Rudiment single hybrid motif |
| Homologous_superfamily | IPR013815 | ATP-grasp fold, subdomain 1 |
| Homologous_superfamily | IPR016185 | Pre-ATP-grasp domain superfamily |
Domain Details Per Protein
| Protein | Length | ATP-grasp fold | ATP-grasp fold, subdomain 1 | Biotin-binding site | Biotin carboxylase, C-terminal | Biotin carboxylase-like, N-terminal domain | Biotin carboxylation domain | Biotin-dependent Carboxylase Complex | Biotin/lipoyl attachment | Carbamoyl-phosphate synthetase large subunit-like, ATP-binding domain | Pre-ATP-grasp domain superfamily | Rudiment single hybrid motif | Single hybrid motif |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
|
UniProtKB:A0A0R4IH35
|
711 |
Interactions and Pathways
No data available
Plasmids
No data available