ATXN2-CAG42 Sequesters PABPC1 into Insolubility and Induces FBXW8 in Cerebellum of Old Ataxic Knock-In Mice
نویسندگان
چکیده
منابع مشابه
ATXN2-CAG42 Sequesters PABPC1 into Insolubility and Induces FBXW8 in Cerebellum of Old Ataxic Knock-In Mice
Spinocerebellar Ataxia Type 2 (SCA2) is caused by expansion of a polyglutamine encoding triplet repeat in the human ATXN2 gene beyond (CAG)(31). This is thought to mediate toxic gain-of-function by protein aggregation and to affect RNA processing, resulting in degenerative processes affecting preferentially cerebellar neurons. As a faithful animal model, we generated a knock-in mouse replacing ...
متن کاملBoth Ubiquitin Ligases FBXW8 and PARK2 Are Sequestrated into Insolubility by ATXN2 PolyQ Expansions, but Only FBXW8 Expression Is Dysregulated
The involvement of the ubiquitin-proteasome system (UPS) in the course of various age-associated neurodegenerative diseases is well established. The single RING finger type E3 ubiquitin-protein ligase PARK2 is mutated in a Parkinson's disease (PD) variant and was found to interact with ATXN2, a protein where polyglutamine expansions cause Spinocerebellar ataxia type 2 (SCA2) or increase the ris...
متن کاملskopos theory and translating strategies of csis in translaed literary texts from english into persian
abstract translation has become a cultural act which plays a significant role in human life. with the emergence of functional translation theories, and skopos theory in particular, translation has been considered as a purposeful, interpersonal and intercultural activity which is produced for particular recipients and directed by a specific purpose. this purpose determines the translatio...
15 صفحه اولan investigation into iranian teachers consistency and bias in evaluation of students writings
while performance-based language assessment has led to an increased authenticity and content validity in the practice of writing assessment, the reliability of ratings has become a major issue. research findings have shown different reactions by native english speaker (nes) and non-native english speaker (nns) teachers to students’ writings. the focus of this study is on investigating whether i...
role of cultural iran in promotion of multilaieralism in central asia and caucasus
cultural iran is a scope that is more extended than the political territories of iran as a political unit. this concept means that cultural geography(mehdi moghanlo-1383-1) of iran is greater than its political geography which, according to history, has a long history extending west-east from kandahar to the euphrates and north-south from the persian gulf to the caucasus including transoxiana a...
15 صفحه اولذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
ژورنال
عنوان ژورنال: PLoS Genetics
سال: 2012
ISSN: 1553-7404
DOI: 10.1371/journal.pgen.1002920