Article
The unfolded protein response has a protective role in yeast models of classic galactosemia.
Disease models & mechanisms - 1 Jan 2014
De-Souza Evandro A, Pimentel Felipe S A, Machado Caio M, Martins Larissa S, da-Silva Wagner S, Montero-Lomelí Mónica, Masuda Claudio A
Abstract excerpt
Classic galactosemia is a human autosomal recessive disorder caused by mutations in the GALT gene (GAL7 in yeast), which encodes the enzyme galactose-1-phosphate uridyltransferase. Here we show that the unfolded protein response pathway is triggered by galactose in two yeast models of galactosemia: lithium-treated cells and the gal7Δ mutant. The synthesis of galactose-1-phosphate is essential to trigger the...
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