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在小鼠中,C9orf72是适当的巨细胞和微细胞功能所需的

J G O'Rourke¹, L Bogdanik², A Yáñez¹

¹Board of Governors Regenerative Medicine Institute, Cedars-Sinai Medical Center, 8700 Beverly Boulevard, Los Angeles, CA 90048, USA.

Science (New York, N.Y.)

|March 19, 2016

概括

C9orf72基因扩展导致ALS和FTD. 在小鼠中C9orf72功能丧失导致免疫细胞问题和神经炎症,这表明神经退行性疾病的作用.

科学领域:

神经科学
遗传学
免疫学

背景情况:

在C9orf72基因中,六核酸重复扩张是肌缩侧面硬化 (ALS) 和前性痴呆 (FTD) 的主要遗传原因.
载体中C9orf72表达的减少表明功能丧失机制有助于疾病的发病.

研究的目的:

研究C9orf72的体内功能及其在神经退行性疾病中的作用.
确定C9orf72功能损失是否导致运动神经元疾病或其他病理.

主要方法:

产生和分析缺少C9orf72的两个独立的小鼠系 (3110043O21Rik).
对C9orf72无细胞小鼠的正常发育,衰老和疾病表型的评估.
分析C9orf72表达模式,特别是在骨髓细胞中.
对C9orf72无细胞小鼠的巨细胞和微质细胞的溶解体功能和免疫反应的研究.
在C9orf72无细胞小鼠和人类ALS患者组织中比较神经炎症.

主要成果:

C9orf72无细胞小鼠的发育正常,没有运动神经元疾病.
观察到渐进的脊髓大和淋巴腺病,其特征是巨细胞的积累.
在骨髓细胞中,C9orf72的表达最高.
失去了C9orf72,导致溶酶体积累,并改变了巨细胞和微质细胞的免疫反应.
在C9orf72无细胞小鼠中检测到与年龄相关的神经炎症,类似于C9orf72相关的ALS,但不是零星ALS.

结论:

C9orf72对于骨髓细胞的正常功能至关重要.
由于C9orf72缺乏而改变的微质功能可能导致C9orf72扩张载体的神经退行.
这项研究强调了C9orf72在ALS和FTD发病过程中的潜在非神经细胞自主作用.

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