Quantitative Evaluation of Toxic Polyglycine Biosynthesis and Aggregation in Cell Models Expressing Expanded CGG Repeats

Fragile X-associated tremor/ataxia syndrome (FXTAS) is a late-onset neurodegenerative disorder caused by expanded CGG (CGGexp) trinucleotides in the 5′UTR of the FMR1 gene encoding fragile X mental retardation protein (FMRP). The patients, with the number of the repeats ranging from 55 to 200, show...

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Egile Nagusiak:	Magdalena Derbis, Patryk Konieczny, Agnieszka Walczak, Michal Sekrecki, Krzysztof Sobczak
Formatua:	Artigo
Hizkuntza:	Inglês
Argitaratua:	Frontiers Media S.A. 2018-06-01
Saila:	Frontiers in Genetics
Gaiak:	FXTAS FXS premutation CGG repeat expansion polyglycine RAN translation
Sarrera elektronikoa:	https://www.frontiersin.org/article/10.3389/fgene.2018.00216/full
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