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Large-scale screen for modifiers of ataxin-3-derived polyglutamine-induced toxicity in Drosophila.

ABSTRACT: Polyglutamine (polyQ) diseases represent a neuropathologically heterogeneous group of disorders. The common theme of these disorders is an elongated polyQ tract in otherwise unrelated proteins. So far, only symptomatic treatment can be applied to patients suffering from polyQ diseases. Despite extensive research, the molecular mechanisms underlying polyQ-induced toxicity are largely unknown. To gain insight into polyQ pathology, we performed a large-scale RNAi screen in Drosophila to identify modifiers of toxicity induced by expression of truncated Ataxin-3 containing a disease-causing polyQ expansion. We identified various unknown modifiers of polyQ toxicity. Large-scale analysis indicated a dissociation of polyQ aggregation and toxicity.

SUBMITTER: VoSSfeldt H

PROVIDER: S-EPMC3489908 | biostudies-literature | 2012

REPOSITORIES: biostudies-literature

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Large-scale screen for modifiers of ataxin-3-derived polyglutamine-induced toxicity in Drosophila.

VoSSfeldt Hannes H Butzlaff Malte M PrüSSing Katja K Ní Chárthaigh Róisín-Ana RA Karsten Peter P Lankes Anne A Hamm Sabine S Simons Mikael M Adryan Boris B Schulz Jörg B JB Voigt Aaron A

PloS one 20121105 11

Polyglutamine (polyQ) diseases represent a neuropathologically heterogeneous group of disorders. The common theme of these disorders is an elongated polyQ tract in otherwise unrelated proteins. So far, only symptomatic treatment can be applied to patients suffering from polyQ diseases. Despite extensive research, the molecular mechanisms underlying polyQ-induced toxicity are largely unknown. To gain insight into polyQ pathology, we performed a large-scale RNAi screen in Drosophila to identify mo ...[more]

PMID: 23139745

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