Mechanism of motor neuron death in ALS: transcription activity of ribosomal RNA gene and TDP-43

本研究旨在阐明肌萎缩侧索硬化症(ALS)患者脊髓运动神经元蛋白质合成系统的异常。这些异常表现为：(1)核糖体(R)RNA基因转录活性降低；(2)rRNA基因活性与核内核仁量相关；(3)分子伴侣聚集；(4)分子伴侣与布尼娜小体中的胱抑素C聚集。

The present study elucidate the abnormalities in the protein synthesis system in the motor neurons in the spinal cords in patients with amyotrophic lateral sclerosis (ALS). Those abnormalities are as follows: 1) decrease of ribosomal (r) RNA gene transcription activity, 2) correlation between rRNA gene activity and amount of nucleolin in the nucleus, 3) aggregation of the molecular chaperone, and 4) seeding each other in aggregation of molecular chaperone and cystatin C, which is a component of the Bunina body.