کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
4351035 1297004 2006 5 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
RNAi blocks DYT1 mutant torsinA inclusions in neurons
موضوعات مرتبط
علوم زیستی و بیوفناوری علم عصب شناسی علوم اعصاب (عمومی)
پیش نمایش صفحه اول مقاله
RNAi blocks DYT1 mutant torsinA inclusions in neurons
چکیده انگلیسی
Early onset generalized dystonia is a dominantly inherited movement disorder caused by neuronal dysfunction without an apparent loss of neurons. The same single mutation (GAG deletion) causes most cases and results in loss of a glutamic acid (E) in the carboxy terminal region of torsinA (Δ302/303). To model the neuronal involvement, adult rat primary sensory dorsal root ganglion neurons in culture were infected with lentivirus vectors expressing human wild-type or mutant torsinA. Expression of the mutant protein resulted in formation of torsinA-positive perinuclear inclusions. When the cells were co-infected with lentivirus vectors expressing the mutant torsinA message and a shRNA selectively targeting this message, inclusion formation was blocked. Vector-delivered siRNAs have the potential to decrease the adverse effects of this mutant protein in neurons without affecting wild-type protein.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Neuroscience Letters - Volume 395, Issue 3, 13 March 2006, Pages 201-205
نویسندگان
, , , , ,