Medical Definition of Methylmalonic aciduria

1. Excretion of excessive amounts of methylmalonic acid in urine owing to deficient activity of methylmalonyl-CoA mutase or deficient cobalamin reductase. Two types occur: 1) an inborn error of metabolism resulting in severe ketoacidosis shortly after birth, with long-chain urinary ketones; autosomal recessive inheritance; 2) acquired, a type due to vitamin B12 deficiency due to defective synthesis of adenosylcobalamin. (05 Mar 2000)

Lexicographical Neighbors of Methylmalonic Aciduria

methylglyoxalase
methylglyoxal dehydrogenase
methylguanidine
methylguanidine synthase
methylhexaneamine
methylhistamines
methylhistidines
methylhydrazines
methylic
methylic
methylkinase
methylmalonate semialdehyde
methylmalonic acid
methylmalonic acidaemia
methylmalonic acidemia
methylmalonic aciduria (current term)
methylmalonyl-CoA
methylmalonyl-CoA epimerase
methylmalonyl-CoA mutase
methylmannosides
methylmercuries
methylmercury
methylmercury
methylmercury compounds
methylmethacrylates
methylmorphine
methylnitronitrosoguanidine
methylnitrosourea
methylococcaceae
methylol

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