The unfolded protein response has a protective role in yeast models of classic galactosemia.
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ABSTRACT:
SUBMITTER: De-Souza EA
PROVIDER: S-EPMC3882048 | biostudies-literature | 2014 Jan
REPOSITORIES: biostudies-literature
Publications
The unfolded protein response has a protective role in yeast models of classic galactosemia.
De-Souza Evandro A EA Pimentel Felipe S A FS Machado Caio M CM Martins Larissa S LS da-Silva Wagner S WS Montero-Lomelí Mónica M Masuda Claudio A CA
Disease models & mechanisms 20130925 1
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 unfolded protein response under these conditions because the deletion of the galactokinas ...[more]
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