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Colorimetric Method for Determination of Urinary Methylmalonic Acid [PDF]
The colorimetric method for the determination of methylmalonic acid by coupling with diazotised p-nitroaniline has been re-examined. Extraction of the diazotised product into amyl alcohol and tetra-methyl ammonium hydroxide considerably increases its stability and sensitivity and reduces background absorbance.
W. Taylor, A. Westwood, G. Davies
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Methylmalonic acid — an endogenous toxin?
Cellular and Molecular Life Sciences, 2005Methylmalonic 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 ...
Jürgen G. Okun, Stefan Kölker
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Methylmalonic Acid in Dialysis Patients
Hemodialysis International, 2003Propionyl-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.
F. Pehal+3 more
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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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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 values in healthy Dutch children
European Journal of Nutrition, 2007Plasma methylmalonic acid (MMA) is a specific marker for functional cobalamin deficiency. This deficiency can give rise to non-specific but serious symptoms in childhood such as developmental delay, convulsions and failure to thrive and may even lead to irreversible neurological damage.To analyse plasma MMA concentrations in Dutch children and to ...
Hogeveen, M.+5 more
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METABOLIC PRECURSORS OF METHYLMALONIC ACID IN VITAMIN-B12 DEFICIENCY
The Lancet, 1967Abstract Valine, isoleucine and sodium propionate have been investigated as precursors of urinary methylmalonic acid in patients with vitamin B12 deficiency. Valine was administered to fifteen patients with vitamin B12 deficiency and provoked an increased excretion of methylmalonic acid in all cases. The same dose of valine had no effect on excretion
J.Hywel Jones, D. Gompertz, J.P. Knowles
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The assay of methylmalonic acid in urine
Clinica Chimica Acta, 1972Abstract The assay of methylmalonic acid in urine by modifications of the method of Giorgio and Plaut 5 , together with the use of a three wavelength correction is described. This revised procedure made possible the detection of small increases in the output of methylmalonic acid in vitamin B 12 deficiency which could not be estimated by the ...
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Kinetics and mechanism for the iodination of methylmalonic acid [PDF]
AbstractThe reaction\documentclass{article}\pagestyle{empty}\begin{document}$ CH\left({CH_3 } \right)\left({COOH} \right)_2 + I_2 \rightleftharpoons CI(CH_3)\left({COOH} \right)_2 + H^ + + I^ - $\end{document} was followed spectrophotometrically at 353 nm and 470 nm at 25°C under various conditions of pH and methylmalonic acid concentration.
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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.
Yin-Hsiu Chien+6 more
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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.
Yin-Hsiu Chien+6 more
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A rapid method for the analysis of urinary methylmalonic acid
Clinica Chimica Acta, 1972Abstract A method for the determination of urinary methylmalonic acid, applicable on a routine clinical basis, is described. The method is based on the isolation of a mono-tooligo-carboxylic acid fraction from urine by a series of ether extractions and the subsequent gas Chromatographie analysis of these acids as their trimethylsilyl esters and ...
K. Itiaba+4 more
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