FIGURE 1.

The proteostasis network and disease disturbances. RNA translation is divided into three steps and controlled by proteostatic processes. Under cellular stress, the assembly of initiation and elongation complexes is inhibited to reduce protein synthesis. Moreover, the premature transcript is degraded by NMD to avoid the generation of aberrant proteins. Mutations in key factors involved in translational procedures induce proteome stress and cause neuronal disorders. Following synthesis, nascent proteins experience posttranslational modifications to achieve their correct folding and become functional. However, misfolded proteins are generated by proteome stress and eliminated by three types of protein degradation mechanisms. Under neuropathies, improper modifications and resistances to degradation attenuate the clearance of misfolded proteins and finally cause the protein aggregation.