Methylmalonyl-CoA mutase
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methylmalonyl Coenzyme A mutase
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| Identifiers | |
| Symbol | MUT |
| HUGO | 7526 |
| Entrez | 4594 |
| OMIM | 609058 |
| RefSeq | NM_000255 |
| UniProt | P22033 |
| Other data | |
| EC number | 5.4.99.2 |
| Locus | Chr. 6 p21 |
Methylmalonyl-Coenzyme A mutase is an enzyme involved in key metabolic pathways. It catalyzes the the isomerization of methylmalonyl-CoA to succinyl-CoA. It requires its [[Vitamin B12]] derived prosthetic group, adenosylcobalamin, to function.
The substrate of Methylmalonyl-CoA mutase, methylmalonyl-CoA, is primarily derived from propionyl-CoA, a substance formed from the catabolism and digestion of isoleucine, valine, threonine, methionine, thymine, uracil, cholesterol, or odd-chain fatty acids.
The product of the enzyme, succinyl-CoA, is a key molecule of the TCA cycle.
A deficiency of this enzyme is responsible for a inherited disorder of metabolism, Methylmalonyl-CoA mutase deficiency.
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