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.

Medical dictionary. 2011.

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  • Méthylmalonyl-coenzyme A —     Structure des énantiomères L (à gauche) e …   Wikipédia en Français

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