Contribution of aminoacid catabolism to propionate production in methylmalonic acidaemia.

Abstract

The rates of propionate production and aminoacid catabolism in 5 children with methylmalonic acidaemia were measured by use of stable isotope techniques. Total propionate production was 55-186 mumol/kg per h, to which the maximum contribution of protein catabolism was 10-35 mumol/kg per h (5-40%). These findings indicate important sources of propionate other than protein catabolism, which may account for the limited efficacy of dietary protein restriction in treatment of methylmalonic acidaemia.

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@article{Walter1989ContributionOA, title={Contribution of aminoacid catabolism to propionate production in methylmalonic acidaemia.}, author={John H. Walter and Geoffrey N Thompson and James V. Leonard and Kim Bartlett and Dave Halliday}, journal={Lancet}, year={1989}, volume={1 8650}, pages={1298-9} }