نتایج جستجو برای: protein misfolding

تعداد نتایج: 1235138  

2010
Daniel W. Neef Michelle L. Turski Dennis J. Thiele

Neurodegenerative diseases such as Huntington disease are devastating disorders with no therapeutic approaches to ameliorate the underlying protein misfolding defect inherent to poly-glutamine (polyQ) proteins. Given the mounting evidence that elevated levels of protein chaperones suppress polyQ protein misfolding, the master regulator of protein chaperone gene transcription, HSF1, is an attrac...

2016
Lisa Ulbrich Flores Lietta Favaloro Laura Trobiani Valentina Marchetti Vruti Patel Tiziana Pascucci Davide Comoletti Stefan J. Marciniak Antonella De Jaco

Several forms of monogenic heritable autism spectrum disorders are associated with mutations in the neuroligin genes. The autism-linked substitution R451C in neuroligin3 induces local misfolding of its extracellular domain, causing partial retention in the ER (endoplasmic reticulum) of expressing cells. We have generated a PC12 Tet-On cell model system with inducible expression of wild-type or ...

2016
James Shorter

Therapeutic agents are urgently required to cure several common and fatal neurodegenerative disorders caused by protein misfolding and aggregation, including amyotrophic lateral sclerosis (ALS), Parkinson's disease (PD), and Alzheimer's disease (AD). Protein disaggregases that reverse protein misfolding and restore proteins to native structure, function, and localization could mitigate neurodeg...

Journal: :Trends in Molecular Medicine 2015

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