C9orf72 repeat expansions cause neurodegeneration in Drosophila through arginine-rich proteins

Autor:	Pietro Fratta, Karen Cleverley, Anny Devoy, Linda Partridge, Oliver Hendrich, Sebastian Grönke, Stuart Pickering-Brown, Andrew J. Nicoll, Frances E. Norona, Julian Pietrzyk, Thomas G. Moens, Ione O.C. Woollacott, Adrian M. Isaacs, Melissa Cabecinha, Elizabeth M. C. Fisher, Emma L. Clayton, Jacqueline Dols, Charlotte E. Ridler, Teresa Niccoli, Sarah Mizielinska
Rok vydání:	2014
Předmět:	Multidisciplinary Dipeptide biology Neurodegeneration RNA Translation (biology) DNA Repeat Expansion Bioinformatics biology.organism_classification medicine.disease Cell biology C9orf72 Protein chemistry.chemical_compound chemistry C9orf72 medicine Drosophila melanogaster
Zdroj:	Science. 345:1192-1194
ISSN:	1095-9203 0036-8075
Popis:	Dipeptide repeat peptides on the attack Certain neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS), are associated with expanded dipeptides translated from RNA transcripts of disease-associated genes (see the Perspective by West and Gitler). Kwon et al. show that the peptides encoded by the expanded repeats in the C9orf72 gene interfere with the way cells make RNA and kill cells. These effects may account for how this genetic form of ALS causes disease. Working in Drosophila , Mizielinska et al. aimed to distinguish between the effects of repeat-containing RNAs and the dipeptide repeat peptides that they encode. The findings provide evidence that dipeptide repeat proteins can cause toxicity directly. Science , this issue p. 1139 and p. 1192 ; see also p. 1118
Databáze:	OpenAIRE
Externí odkaz:	https://explore.openaire.eu/search/publication?articleId=doi_________::65038fd02fb5c543aac4a3d6b4ef83f8 https://doi.org/10.1126/science.1256800 Zobrazit plný text záznamu Plný text ve formátu PDF