Scientific Reports：日发现一种蛋白质或能推迟渐冻症发展

日本岐阜药科大学教授原英彰率领的研究小组在8月13日的英国《科学报告》（Scientific Reports）杂志网络版上报告说，他们发现了一种能推迟渐冻症发展的蛋白质。这一发现有望为弄清该病的发病机制和开发新的治疗药物作出贡献。

肌萎缩侧索硬化症俗称渐冻症，这种疾病的患者运动神经出现障碍，导致全身肌肉逐渐变得无力。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，医学界一直未找到根治此病的方法。

研究小组在对渐冻症实验鼠和正常实验鼠进行比较时，发现渐冻症实验鼠体内的“膜贯通糖蛋nmb”（GPNMB）与病情的发展有很大关系。

研究人员通过基因操作，使十几只渐冻症实验鼠体内的GPNMB得以大量表达，结果发现与对照组的渐冻症实验鼠相比，前者的发病时间推迟，生存时间也延长了1至２周左右，换算成人类，相当于生命延长了10个月左右，从而证实GPNMB能够推迟渐冻症发展。

名古屋大学研究生院、新潟大学等机构的研究人员也参与了此项研究。

doi:10.1038/srep00530
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The Bcl-2 family member BIM has multiple glaucoma-relevant functions in DBA/2J mice

Jeffrey M. Harder; Kimberly A. Fernandes; Richard T. Libby

Axonal insult induces retinal ganglion cell (RGC) death through a BAX-dependent process. The pro-apoptotic Bcl-2 family member BIM is known to induce BAX activation. BIM expression increased in RGCs after axonal injury and its induction was dependent on JUN. Partial and complete Bim deficiency delayed RGC death after mechanical optic nerve injury. However, in a mouse model of glaucoma, DBA/2J mice, Bim deficiency did not prevent RGC death in eyes with severe optic nerve degeneration. In a subset of DBA/2J mice, Bim deficiency altered disease progression resulting in less severe nerve damage. Bim deficient mice exhibited altered optic nerve head morphology and significantly lessened intraocular pressure elevation. Thus, a decrease in axonal degeneration in Bim deficient DBA/2J mice may not be caused by a direct role of Bim in RGCs. These data suggest that BIM has multiple roles in glaucoma pathophysiology, potentially affecting susceptibility to glaucoma through several mechanisms.