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Literature summary for 6.4.1.4 extracted from
- Molecular mechanism of dominant expression in 3-methylcrotonyl-CoA carboxylase deficiency (2005), J. Inherit. Metab. Dis., 28, 301-309.
Protein Variants
| Protein Variants | Comment | Organism |
|---|---|---|
| R385S | mutant leading to biochemical abnormalities and clinical symptoms in heterozygous individuals | Homo sapiens |
Localization
| Localization | Comment | Organism | GeneOntology No. | Textmining |
|---|---|---|---|---|
| mitochondrion | - |
Homo sapiens | 5739 | - |
Natural Substrates/ Products (Substrates)
| Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| ATP + 3-methylcrotonyl-CoA + HCO3- | Homo sapiens | - |
ADP + 3-methylglutaconyl-CoA + phosphate | - |
? |  |
Organism
| Organism | UniProt | Comment | Textmining |
|---|---|---|---|
| Homo sapiens | - |
- |
- |
Source Tissue
| Source Tissue | Comment | Organism | Textmining |
|---|---|---|---|
| fibroblast | - |
Homo sapiens | - |
Substrates and Products (Substrate)
| Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| ATP + 3-methylcrotonyl-CoA + HCO3- | - |
Homo sapiens | ADP + 3-methylglutaconyl-CoA + phosphate | - |
? | |
Subunits
| Subunits | Comment | Organism |
|---|---|---|
| heteromer | alphabeta | Homo sapiens |
Synonyms
| Synonyms | Comment | Organism |
|---|---|---|
| MCC | - |
Homo sapiens |
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Release 2023.1 (January 2023)
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