methylmalonyl-CoA mutase

methylmalonyl-CoA mutase
meth·yl·mal·o·nyl-CoA mu·tase (meth″əl-malґə-nəl ko-aґ muґtās) [EC 5.4.99.2] an enzyme of the isomerase class that catalyzes the isomerization of L-methylmalonyl coenzyme A to succinyl coenzyme A, requiring adenosylcobalamin as a coenzyme. The reaction is a step in the use of isoleucine, threonine, valine, propionate, and other odd-number chain length fatty acids as fuels. Deficiency of enzyme activity, which may be caused by defects in the apoenzyme, in the coenzyme, or in cobalamin metabolism, results in methylmalonicacidemia.

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