Hum Mol Genet:肌萎缩性脊髓侧索硬化症(渐冻症)可能与ter94基因有关

2014-05-03 MedSci MedSci原创

肌萎缩侧索硬化症俗称渐冻症,患者运动神经会出现障碍,导致全身肌肉逐渐变得无力。日本研究人员日前宣布,他们发现一种蛋白质能够改善运动障碍等症状,有望在此基础上开发出治疗渐冻症的方法。   渐冻症患者约有10%属于遗传性患病。由于不清楚详细的致病原因,医学界一直没有找到根治渐冻症的方法。     日本京都府立医科大学和京都工艺纤维大学的研究人员通过减弱

肌萎缩侧索硬化症俗称渐冻症,患者运动神经会出现障碍,导致全身肌肉逐渐变得无力。日本研究人员日前宣布,他们发现一种蛋白质能够改善运动障碍等症状,有望在此基础上开发出治疗渐冻症的方法。
  
渐冻症患者约有10%属于遗传性患病。由于不清楚详细的致病原因,医学界一直没有找到根治渐冻症的方法。
   
日本京都府立医科大学和京都工艺纤维大学的研究人员通过减弱特定基因的功能,培育出患有渐冻症的果蝇,然后利用这种果蝇进行实验。结果发现,如果增强患病果蝇体内“ter94”基因的功能,其运动能力和神经细胞的异常会得到改善,而如果减弱这种基因的功能,其渐冻症症状就会恶化。
  
研究人员指出,“ter94”基因制造的蛋白质负责运送细胞内的物质,而人体内的VCP基因也能制造同样的蛋白质。因此,如果制造出与这种蛋白质具有相同作用的物质,该物质也许可以用作治疗人类渐冻症的药物。

原始出处:
Azuma Y, Tokuda T, Shimamura M, Kyotani A, Sasayama H, Yoshida T, Mizuta I, Mizuno T, Nakagawa M, Fujikake N, Ueyama M, Nagai Y, Yamaguchi M.Identification of ter94, Drosophila VCP, as a strong modulator of motor neuron degeneration induced by knockdown of Caz, Drosophila FUS.Hum Mol Genet. 2014 Feb 18.

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    2014-08-19 canlab
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    2015-02-05 cy0324
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    2014-12-17 chendoc252
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    2014-05-05 xjy02
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