Unfolded Protein Response and Activated Degradative Pathways Regulation in GNE Myopathy

来源 :The 12th Annual Asian and Oceanian Myology Center (AOMC)Scie | 被引量 : 0次 | 上传用户：jiejie2717

【摘要】

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【作者】

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Honghao Li Qi Chen Fuchen Liu Wei Li Shuping Liu Yuying Zhao Yaoqin Gong Xuemei Zhang Chuan zhu Yan

【机构】

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Department of Neurology, Qilu Hospital of Shandong University, No.107 West Wenhua Road, Jinan 250012

【出处】

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The 12th Annual Asian and Oceanian Myology Center (AOMC)Scie

【发表日期】

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2013年7期

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Objective Although intracellular beta amyloid (Aβ)accumulation is known as an early upstream event in the degenerative course of UDP-N-acetylglucosamine2-epimerase/N-acetylmannosamine kinase(GNE)myopathy,the process by which Aβdeposits initiate various degradative pathways, and their relationship have not been fully clarified.We studied the possible secondary responses after amyloid beta precursor protein (AβPP) deposition including unfolded protein response(UPR),ubiquitin proteasome system(UPS) activation and its correlation with autophagy system.

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Unfolded Protein Response and Activated Degradative Pathways Regulation in GNE Myopathy

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