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Lancet. 1989 Jun 10;1(8650):1298-9.

Contribution of aminoacid catabolism to propionate production in methylmalonic acidaemia.

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1
Department of Child Health, Institute of Child Health, London.

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.

PMID:
2566828
[Indexed for MEDLINE]

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