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.

Игры ⚽ Поможем сделать НИР

Look at other dictionaries:

  • Methylmalonyl-CoA mutase — Rendering based on PDB 2XIJ …   Wikipedia

  • methylmalonyl-CoA mutase — methylmalonyl CoA mutase. См. метилмалонил КоА мутаза. (Источник: «Англо русский толковый словарь генетических терминов». Арефьев В.А., Лисовенко Л.А., Москва: Изд во ВНИРО, 1995 г.) …   Молекулярная биология и генетика. Толковый словарь.

  • Methylmalonyl-CoA-Mutase — Humane L Methylmalonyl CoA Mutase Bändermodell nach PDB …   Deutsch Wikipedia

  • Methylmalonyl-CoA mutase deficiency — Classification and external resources OMIM 251000 DiseasesDB 29509 Methylmalonyl CoA mutase deficiency ( MUT ) is an inborn error of organ …   Wikipedia

  • L-Methylmalonyl-CoA-Mutase — Humane L Methylmalonyl CoA Mutase Bändermodell nach PDB  …   Deutsch Wikipedia

  • Methylmalonyl-CoA — Identifiers CAS number 1264 45 5 …   Wikipedia

  • Methylmalonyl CoA epimerase — Ribbon diagram of methylmalonyl CoA epimerase from Propionibacterium shermanii. From PDB 1JC5 …   Wikipedia

  • Methylmalonyl-CoA — Strukturformel Allgemeines Name Methylmalonyl CoA Summenformel …   Deutsch Wikipedia

  • methylmalonyl-CoA — An intermediate in the degradation of several metabolites ( e.g., valine, methionine, odd chain fatty acid s, theronine); elevated in cases of pernicious anemia. methylmalonyl CoA epimerase an enzyme that catalyzes the interconversion …   Medical dictionary

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

Share the article and excerpts

Direct link
… Do a right-click on the link above
and select “Copy Link”