Oxysterol-binding protein ORP3 rescues the Amyotrophic Lateral Sclerosis-linked mutant VAPB phenotype
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- 作者：Angie Darbyson ; Johnny K. Ngsee ; jngsee@uottawa.ca" class="auth_mail" title="E-mail the corresponding author
- 关键词：ALS ; Amyotrophic Lateral Sclerosis ; ER ; endoplasmic reticulum ; ERGIC ; endoplasmic reticulum Golgi intermediate compartment ; FFAT ; two phenylalanines in an acidic tract ; NE ; Nuclear envelope ; ORP ; Oxysterol binding protein-related protein ; PtdIns4KIII ; PtdIns4-kinase type III ; PtdIns4P ; Phosphatidylinositol 4-phosphate ; VAPB ; Vesicle associated membrane protein associated protein B
- 刊名：Experimental Cell Research
- 出版年：2016
- 出版时间：1 February 2016
- 年：2016
- 卷：341
- 期：1
- 页码：18-31
- 全文大小：5545 K
文摘
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Co-overexpression of ORP3 rescues the mutant VAPB aggregation phenotype.
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It restores mutant VAPB-induced protein trafficking defect.
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VAPB and ORP3 collectively regulate intracellular PtdIns4P levels.
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PtdIns4P plays a pivotal role in this VAPB-mediated protein trafficking step.