WebWith conventional dietary treatment, the clinical course of methylmalonic acidemia due to cobalamin-unresponsive methylmalonyl-CoA mutase (MCM) deficiency is characterized by the persistent risk of recurrent life-threatening decompensation episodes Web10 feb. 2024 · Methylmalonyl coenzyme A mutase (MCM) catalyzes the adenosylcobalamin-dependent isomerization of methylmalonyl-CoA to succinyl-CoA. Adenosyltransferase, an enzyme that carries out the...
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Web8 mrt. 2024 · Control over transition metal redox state is essential for metalloprotein function and can be achieved via coordination chemistry and/or sequestration from bulk solvent. Human methylmalonyl-Coenzyme A (CoA) mutase (MCM) catalyzes the isomerization of methylmalonyl-CoA to succinyl-CoA using 5'-deoxyadenosylcobalamin … Web• Catalyzed by methylmalonyl-CoA mutase, • 5'-deoxyadenosylcobalamin' as coenzyme Propionic Acidemia • Propionyl-CoA carboxylase ... Infantile Refsum disease • Ataxia &broad based gait • Impaired Cognitive function • Sensorineural hearing loss • Pigmentary degeneration of the retina. • Dysmorphic features -Epicanthal ... boston wood floor supply dorchester
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Web4 mei 1999 · Catalyzes the reversible isomerization of methylmalonyl-CoA (MMCoA) (generated from branched-chain amino acid metabolism and degradation of dietary odd chain fatty acids and cholesterol) to succinyl-CoA (3-carboxypropionyl-CoA), a key intermediate of the tricarboxylic acid cycle. • gold 10 ev UniProt ENZYMATIC ACTIVITY WebAdenosylcobalamin (Adocbl) is the cofactor for methylmalonyl-CoA mutase, which converts methylmalonylCoA into succinyl-CoA in mitochondria (Figure 2). WebMMA: methylmalonic acid; MUT: methylmalonyl CoA mutase; Mut -: partial loss of MUT function; Mut 0: complete loss of MUT function; Adocbl: adenosylcobalamin; cbl: cobalamin; MMAA:... hawks x y/n fanfiction