Possible Explanation for Hyperglycinaemia in Propionic Acidaemia and Methylmalonic Acidaemia: Propionate and Methylmalonate Inhibit Liver and Brain Mitochondrial Clycine Transport

Overview

Journal J Inherit Metab Dis

Publisher Wiley

Date 1980 Jan 1

PMID 6796767

Citations 1

Authors

M Ugarte

M L Garcia

J Benavides

F Valdivieso

Affiliations

Soon will be listed here.

Abstract

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 14CO2 production from [14C]glycine by isolated brain and liver mitochondria and glycine metabolism in rat brain cortex slices. These results are discussed with reference to hyperglycinaemia associated wtih propionic acidaemia and methylmalonic acidaemia and as a possible contributory factor to the pathological mechanisms in these conditions.

Citing Articles

Stimulation of ketogenesis by propionate in isolated rat hepatocytes: an explanation for ketosis associated with propionic acidaemia and methylmalonic acidaemia?.

Rosario P, Medina J J Inherit Metab Dis. 1982; 5(1):59-62.

PMID: 6820416 DOI: 10.1007/BF01799756.

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