Methylmalonyl coenzyme A epimerase

Table 9.1 Test data methylmalonyl-coenzyme A epimerase from Propionibacterium shermanii...

McCarthy, A. A., Baker, H. M., Shewry S. C., Patchett, M. L. and Baker, E. N. (2001). Crystal structure of methylmalonyl-coenzyme A epimerase from P. sher-manii a novel enzymatic function on an ancient metal binding scaffold. Structure 9,637-646. 

R) -2-Methyl-3-oxopropanoyl-coenzyme A, METHYLMALONYL-CoA EPIMERASE l-METHYLMALONYL-CoA MUTASE... 

Methylmalonyl-CoA mutase (EC 5.4.99.2). Failure to convert (/ )-methylmalonyl-CoA into succinyl-CoA. Large quantities of methylmalonic acid appear in plasma and urine. Affected children fail to thrive and show pronounced ketoacidosis. Often fatal in early life. Hyperammonemia and intermittent hyperglycinemia are also typical. Restricted protein intake and synthetic diets are helpful, in particular low intakes of leucine, isoleucine, valine, threonine and methionine. A similar condition may arise from a congenital deficiency of methylmalonyl-CoA epimerase (EC 5.1.99.1). Both conditions unresponsive to vitamin Bj2. Another type of methylmalonyl aciduria is thought to result from an hereditary deficiency of deoxyadenosyl transferase (transfers the 5 -deoxyade-nosyl group in cobalamin synthesis), which provides the coenzyme of methylmalonyl-CoA mutase. This condition responds to injection of B,2. Dietary B12 deficiency also results in methylmalonic aciduria. 

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