Modeling Protein Misfolding in Charcot–Marie–Tooth Disease

摘要: Charcot–Marie–Tooth (CMT) disease is the most common inherited neuromuscular disorder. Recent advancements in molecular biology have elucidated bases of this genetically heterogeneous neuropathy. Still, major challenge lies determining individual contributions by malfunctions proteins to disease’s pathology. This paper reviews identified mechanisms underlying forms CMT disease. A growing body evidence has highlighted role protein misfolding demyelinating peripheral neuropathies and neurodegenerative diseases. Several hypotheses been proposed explain how misfolded aggregates induce neuronal damage. Current research focuses on developing novel therapeutic targets which aim prevent, or even reverse formation aggregation. Interestingly, cellular defence against accumulation may play a key leading strategies for treatment accelerating clearance their toxic early aggregates. Based these findings we propose model describing terms formal computer language, biomolecular processes involving associated with