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Possible explanation for hyperglycinaemia in propionic acidaemia and methylmalonic acidaemia: Propionate and methylmalonate inhibit liver and brain mitochondrial glycine transport
Author(s) -
Ugarte M.,
LopezLahoya J.,
Garcia M. L.,
Benavides J.,
Valdivieso F.
Publication year - 1979
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1007/bf01805665
Subject(s) - propionate , glycine , methylmalonic acid , chemistry , mitochondrion , glycine cleavage system , metabolism , biochemistry , endocrinology , amino acid , biology , homocysteine
The effect of propionate and methylmalonate on the transport of glycine into rat liver and brain mitochondria was investigated. Both propionate and methylmalonate markedly inhibited mitochondrial glycine transport. These compounds also inhibited 14 CO 2 production from [ 14 C]glycine by isolated brain and liver mitochondria and glycine metabolism in rat brain cortex slices. These results are discussed with reference to hyperglycinaemia associated with propionic acidaemia and methylmalonic acidaemia and as a possible contributory factor to the pathological mechanisms in these conditions.