Pathogenic Mutations in the Valosin-containing Protein/p97(VCP) N-domain Inhibit the SUMOylation of VCP and Lead to Impaired Stress Response.

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第一作者： Tao,Wang

第一单位： Institute of Neuroscience, State Key Laboratory of Neuroscience, Key Laboratory of Primate Neurobiology, Shanghai Institutes for Biological Sciences, Chinese Academy of Sciences, Shanghai 200031.

作者： Tao,Wang ^[1] ; Wangchao,Xu ^[2] ; Meiling,Qin ^[1] ; Yi,Yang ^[2] ; Puhua,Bao ^[1] ; Fuxiao,Shen ^[1] ; Zhenlin,Zhang ^[3] ; Jin,Xu ^[4]

作者单位： Institute of Neuroscience, State Key Laboratory of Neuroscience, Key Laboratory of Primate Neurobiology, Shanghai Institutes for Biological Sciences, Chinese Academy of Sciences, Shanghai 200031. ^[1] Institute of Neuroscience, State Key Laboratory of Neuroscience, Key Laboratory of Primate Neurobiology, Shanghai Institutes for Biological Sciences, Chinese Academy of Sciences, Shanghai 200031,; University of Chinese Academy of Sciences, Shanghai 200031, China. ^[2] Department of Osteoporosis and Bone Diseases, Metabolic Bone Disease and Genetic Research Unit, Shanghai Jiao Tong University Affiliated People's No.6 Hospital, Shanghai 200233, China. ^[3] Institute of Neuroscience, State Key Laboratory of Neuroscience, Key Laboratory of Primate Neurobiology, Shanghai Institutes for Biological Sciences, Chinese Academy of Sciences, Shanghai 200031,. Electronic address: Jin.Xu@ion.ac.cn. ^[4]

关键词 amyotrophic lateral sclerosis (ALS) (Lou Gehrig disease)endoplasmic reticulum-associated protein degradation (ERAD)hexamer post-translational modification (PTM)stress granule stress response sumoylation valosin-containing protein/p97 (VCP)