Large-Scale Screen for Modifiers of Ataxin-3-Derived Polyglutamine-Induced Toxicity in Drosophila

H Voßfeldt, M Butzlaff, K Prüßing, RA Ní Chárthaigh… - PLoS …, 2012 - journals.plos.org
Polyglutamine (polyQ) diseases represent a neuropathologically heterogeneous group of
disorders. The common theme of these disorders is an elongated polyQ tract in otherwise …

Caenorhabditis elegans as a model system for discovering bioactive compounds against polyglutamine-mediated neurotoxicity

Q Wang, J Zhang, Y Jiang, Y Xiao, X Li, X Mao… - JoVE (Journal of …, 2021 - jove.com
Age-related misfolding and aggregation of pathogenic proteins are responsible for several
neurodegenerative diseases. For example, Huntington's disease (HD) is principally driven …

Aggregate formation inhibits proteasomal degradation of polyglutamine proteins

LGGC Verhoef, K Lindsten, MG Masucci… - Human molecular …, 2002 - academic.oup.com
Insoluble protein aggregates are consistently found in neurodegenerative disorders caused
by expanded polyglutamine [poly (Q)] repeats. The aggregates contain various components …

Polyglutamine expansion accelerates the dynamics of ataxin-1 and does not result in aggregate formation

HA Krol, PM Krawczyk, KS Bosch, JA Aten, EM Hol… - PLoS …, 2008 - journals.plos.org
Background Polyglutamine expansion disorders are caused by an expansion of the
polyglutamine (polyQ) tract in the disease related protein, leading to severe …

High‐Throughput Screen of Natural Product Extracts in A Yeast Model of Polyglutamine Proteotoxicity

GM Walter, A Raveh, SA Mok… - Chemical biology & …, 2014 - Wiley Online Library
Proteins with expanded polyglutamine (poly Q) segments cause a number of fatal
neurodegenerative disorders, including Huntington's disease (HD). Previous high …

Nanoscale studies link amyloid maturity with polyglutamine diseases onset

FS Ruggeri, S Vieweg, U Cendrowska, G Longo… - Scientific reports, 2016 - nature.com
The presence of expanded poly-glutamine (polyQ) repeats in proteins is directly linked to the
pathogenesis of several neurodegenerative diseases, including Huntington's disease …

Kaleidoscopic protein–protein interactions in the life and death of ataxin-1: new strategies against protein aggregation

C De Chiara, A Pastore - Trends in neurosciences, 2014 - cell.com
Understanding how proteins protect themselves from aberrant aggregation is of primary
interest for understanding basic biology, protein biochemistry, and human disease. We …

Mutant Ataxin-3–Containing Aggregates (MATAGGs) in Spinocerebellar Ataxia Type 3: Dynamics of the Disorder

K Raj, RS Akundi - Molecular neurobiology, 2021 - Springer
Abstract Spinocerebellar ataxia type 3 (SCA3) is the most common type of SCA worldwide
caused by abnormal polyglutamine expansion in the coding region of the ataxin-3 gene …

Polyglutamine misfolding in yeast: toxic and protective aggregation

ML Duennwald - Prion, 2011 - Taylor & Francis
Protein misfolding is associated with many human diseases, including neurodegenerative
diseases, such as Alzheimer disease, Parkinson disease and Huntington disease. Protein …

Toxicity and aggregation of the polyglutamine disease protein, ataxin-3 is regulated by its binding to VCP/p97 in Drosophila melanogaster

G Ristic, JR Sutton, K Libohova, SV Todi - Neurobiology of Disease, 2018 - Elsevier
Among the nine dominantly inherited, age-dependent neurodegenerative diseases caused
by abnormal expansion in the polyglutamine (polyQ) repeat of otherwise unrelated proteins …