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Merck

Deciphering the glycosylome of dystroglycanopathies using haploid screens for lassa virus entry.

Science (New York, N.Y.) (2013-03-23)
Lucas T Jae, Matthijs Raaben, Moniek Riemersma, Ellen van Beusekom, Vincent A Blomen, Arno Velds, Ron M Kerkhoven, Jan E Carette, Haluk Topaloglu, Peter Meinecke, Marja W Wessels, Dirk J Lefeber, Sean P Whelan, Hans van Bokhoven, Thijn R Brummelkamp
ABSTRACT

Glycosylated α-dystroglycan (α-DG) serves as cellular entry receptor for multiple pathogens, and defects in its glycosylation cause hereditary Walker-Warburg syndrome (WWS). At least eight proteins are critical to glycosylate α-DG, but many genes mutated in WWS remain unknown. To identify modifiers of α-DG, we performed a haploid screen for Lassa virus entry, a hemorrhagic fever virus causing thousands of deaths annually that hijacks glycosylated α-DG to enter cells. In complementary screens, we profiled cells for absence of α-DG carbohydrate chains or biochemically related glycans. This revealed virus host factors and a suite of glycosylation units, including all known Walker-Warburg genes and five additional factors critical for the modification of α-DG. Our findings accentuate the complexity of this posttranslational feature and point out genes defective in dystroglycanopathies.