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BBS4 Is Essential for Nuclear Transport of Transcription Factors Mediating Neuronal ER Stress Response

Mol Neurobiol. 2020; 
Avital Horwitz, Ruth Birk
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Molecular Biology Reagents … Short-Interfering RNA-Mediated Knock-Down. Constructs were designed to express short hairpin-interfering RNA (shiRNA) and were cloned by GenScript into constative-expressing GFP p.RANT-H1/neo vector (Genscript, USA) previously in our lab [11] … Get A Quote

摘要

Bardet-Biedl syndrome (BBS) is an autosomal recessive syndrome presenting with retinal dystrophy, cognitive impairment, and obesity. BBS is characterized by elevated endoplasmic reticulum (ER) stress in the early stages of adipocyte and retinal development. BBS expression in the CNS and indications of hippocampal dysgenesis suggest neural development abnormalities. However, the role of BBS in ER stress in neuronal cells has not yet been studied. Therefore, we aimed at studying the role of BBS4 in neuronal development under normal and ER stress conditions. ER stress and unfolded protein response (UPR) were studied in BBS4-silenced (SiBBS4) SH-SY5Y cells during differentiation under normal and stress states, usin... More

关键词

ATF6α, BBS4, Endoplasmic reticulum (ER), Unfolded protein response (UPR), XBP-1