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The involvement of glial cells in FUS-related ALS/FTLD pathophysiology

  • P2-312
  • 藤岡 祐介 / Yusuke Fujioka:1 石垣 診祐 / Shinsuke Ishigaki:1 白水 崇 / Takashi Shiromizu:2 宇田川 剛 / Tsuyoshi Udagawa:1 横井 聡 / Satoshi Yokoi:1 本田 大祐 / Daiyu Honda:1 池中 健介 / Kensuke Ikenaka:1 勝野 雅央 / Masahisa Katsuno:1 朝長 毅 / Takeshi Tomonaga:2 祖父江 元 / Gen Sobue:1 
  • 1:名古屋大院医神経内科 / Dept Neurol, Univ of Nagoya, Nagoya, Japan 2:医薬基盤研究所 / National Institute of Biomedical Innovation, Osaka, Japan 

Amyotrophic lateral sclerosis (ALS) is one of the most common neurodegenerative disorders, characterized by selective motor neuron degeneration in the primary motor cortex, brainstem and spinal cord. FUS is the causative gene of familial ALS and pathologically linked to sporadic ALS and frontotemporal lobar degeneration (FTLD). FUS is an RNA binding protein which was reported to function in transcription, RNA splicing, and RNA transport in various cells including neurons and glial cells. Recently, non-cell autonomous toxicity has been thought to be involved in the pathophysiology of SOD1-related ALS. However, it has not been clear whether glial cells have effects on neurons in FUS-related ALS/FTLD pathology. To elucidate the effects of FUS-silenced glial cells to neurons, the neurite outlength of primary cortical neurons (PCN) co-cultured with glial cells with/without FUS was measured by the autoimage scanner. Surprisingly, the neurite outlength of PCN were elongated after the co-culture with FUS knock-down glial cells, indicating that FUS knock-down glial cells have a protective effect. Next, we identified some proteins specifically elevated in the culture supernatant of FUS-silenced glial cells using LC-MS/MS analysis as candidates for protective factors. Thus, glial cells which lost the function of FUS countervail the progression of the pathology of FUS related ALS/FTLD through the expression of protective factors.

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