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Methylmalonic Acid Excretion Studies

British Journal of Haematology, 1973
Summary. Oral valine and isoleucine were equally effective in producing an increased urinary excretion of methylmalonic acid (MMA) in vitamin B12 deficiency but oral MMA itself, methionine and leucine were unsatisfactory.MMA excretion was increased in 16 out of 23 patients with vitamin B12 deficiency after valine and/or isoleucine loading but remained
I, Chanarin   +3 more
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Serum methylmalonic acid in uraemia

Scandinavian Journal of Clinical and Laboratory Investigation, 1992
During a 3 month period we measured serum methylmalonic acid concentrations monthly in 37 patients, all on chronic haemodialysis because of end-stage kidney disease. Concentrations of methylmalonic acid in serum were above the upper reference limit in 36 of the 37 subjects.
L, Moelby, K, Rasmussen, H H, Rasmussen
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Methylmalonic acid — an endogenous toxin?

Cellular and Molecular Life Sciences, 2005
Methylmalonic acid was previously considered as major neurotoxin in methylmalonic acidurias. In contrast, recent studies support the notion that other metabolites deriving from propionyl-coenzyme A, inducing synergistic inhibition of mitochondrial energy metabolism, are more important than methylmalonic acid to understand the neuropathogenesis of this ...
S, Kölker, J G, Okun
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METHYLMALONIC ACID

Pediatrics, 1973
Methylmalonate studies have led to some understanding of vitamin B12 metabolism as well as certain inborn errors of metabolism. These, in turn, have served as models of a group of diseases related to acidosis, so that the study of organic aciduria at present is similar to that of amino aciduria 20 years ago.
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Methylmalonic Acid in Dialysis Patients

Hemodialysis International, 2003
Propionyl-CoA is a common product of metabolism of essential AA (Val, Thr, Leu, Ile), fatty acids and cholesterol and is further metabolised enzymatically to methyl-malonyl CoA, which is further isomerised to succinyl CoA. Because of the common cofactor (cobalamin) methylmalonic acid (Mma) can serve as an early marker of functional cobalamin deficiency.
L. Svoboda   +3 more
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Stabilization of blood methylmalonic acid level in methylmalonic acidemia after liver transplantation

Pediatric Transplantation, 2010
Methylmalonic acidemia with complete mutase deficiency (mut(0) type) is an inborn error of metabolism with high mortality and morbidity. LT has been suggested to be a solution to this disease, but elevation of urinary and blood MMA was still observed after LT.
P W, Chen   +6 more
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