Sami Neha, Rahman Safikur, Kumar Vijay, Zaidi Sobia, Islam Asimul, Ali Sher, Ahmad Faizan, Hassan Md Imtaiyaz
a Centre for Interdisciplinary Research in Basic Sciences , Jamia Millia Islamia , New Delhi , India.
b Department of Medical Biotechnology , Yeungnam University , Gyeongsan , South Korea.
Int J Neurosci. 2017 Nov;127(11):1047-1057. doi: 10.1080/00207454.2017.1286339. Epub 2017 Feb 8.
Proteins are major components of the biological functions in a cell. Biology demands that a protein must fold into its stable three-dimensional structure to become functional. In an unfavorable cellular environment, protein may get misfolded resulting in its aggregation. These conformational disorders are directly related to the tissue damage resulting in cellular dysfunction giving rise to different diseases. This way, several neurodegenerative diseases such as Alzheimer, Parkinson Huntington diseases and amyotrophic lateral sclerosis are caused. Misfolding of the protein is prevented by innate molecular chaperones of different classes. It is envisaged that work on this line is likely to translate the knowledge into the development of possible strategies for early diagnosis and efficient management of such related human diseases. The present review deals with the human neurodegenerative diseases caused due to the protein misfolding highlighting pathomechanisms and therapeutic intervention.
蛋白质是细胞生物学功能的主要组成部分。生物学要求蛋白质必须折叠成其稳定的三维结构才能发挥功能。在不利的细胞环境中，蛋白质可能会错误折叠，导致其聚集。这些构象紊乱与组织损伤直接相关，导致细胞功能障碍，引发不同疾病。通过这种方式，引发了几种神经退行性疾病，如阿尔茨海默病、帕金森病、亨廷顿病和肌萎缩侧索硬化症。不同类别的天然分子伴侣可防止蛋白质错误折叠。可以设想，这方面的工作可能会将相关知识转化为针对此类人类相关疾病的早期诊断和有效管理的可能策略。本综述探讨了由蛋白质错误折叠引起的人类神经退行性疾病，重点介绍了发病机制和治疗干预措施。
