Program in Developmental Biology, Baylor College of Medicine, Houston, TX, USA.
Curr Opin Neurobiol. 2011 Feb;21(1):182-8. doi: 10.1016/j.conb.2010.08.014. Epub 2010 Sep 9.
The Drosophila neuromuscular junction (NMJ) has recently provided new insights into the roles of various proteins in neurodegenerative diseases including Amyotrophic Lateral Sclerosis (ALS), Spinal Muscular Atrophy (SMA), Multiple Sclerosis (MS) Hereditary Spastic Paraplegia (HSP), and Huntington's Disease (HD). Several developmental signaling pathways including WNT, MAPK and BMP/TGF-β signaling play important roles in the formation and growth of the Drosophila NMJ. Studies of the fly homologues of genes that cause neurodegenerative disease at the NMJ have resulted in a better understanding of the roles of these proteins in vivo. These studies may shed light on the pathological mechanisms of these diseases, with implications for reduced BMP/TGF-β signaling in ALS, SMA and HD and increased signaling in HSP and MS.
果蝇的神经肌肉接头(NMJ)最近为各种蛋白质在神经退行性疾病中的作用提供了新的见解,包括肌萎缩侧索硬化症(ALS)、脊髓性肌萎缩症(SMA)、多发性硬化症(MS)、遗传性痉挛性截瘫(HSP)和亨廷顿病(HD)。包括 WNT、MAPK 和 BMP/TGF-β 信号在内的几种发育信号通路在果蝇 NMJ 的形成和生长中发挥着重要作用。对 NMJ 处引起神经退行性疾病的基因的果蝇同源物的研究,使人们更好地理解了这些蛋白质在体内的作用。这些研究可能揭示这些疾病的病理机制,提示 ALS、SMA 和 HD 中 BMP/TGF-β 信号减少,而 HSP 和 MS 中信号增加。