RRC ID 21842
Author VoSSfeldt H, Butzlaff M, PrüSSing K, Ní Chárthaigh RA, Karsten P, Lankes A, Hamm S, Simons M, Adryan B, Schulz JB, Voigt A.
Title Large-scale screen for modifiers of ataxin-3-derived polyglutamine-induced toxicity in Drosophila.
Journal PLoS One
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.
Volume 7(11)
Pages e47452
Published 2012-11-5
DOI 10.1371/journal.pone.0047452
PII PONE-D-12-03063
PMID 23139745
PMC PMC3489908
MeSH Animals Ataxin-3 Computational Biology Drosophila Proteins / chemistry Drosophila Proteins / metabolism* Drosophila melanogaster / drug effects* Drosophila melanogaster / metabolism* Models, Biological Nerve Tissue Proteins / chemistry Nerve Tissue Proteins / metabolism* Nuclear Proteins / chemistry Nuclear Proteins / metabolism* Peptides / chemistry Peptides / toxicity* Protein Structure, Quaternary RNA Interference* Repressor Proteins / chemistry Repressor Proteins / metabolism* Retina / drug effects Retina / pathology
IF 2.74
Times Cited 14
WOS Category BIOCHEMISTRY & MOLECULAR BIOLOGY
Resource
Drosophila