Proline to the rescue

摘要

Protein misfolding is now recognized to be a major contributing factor in a number of protein folding diseases, including amyo-tropic laterial sclerosis, cystic fibrosis, Alzheimer's disease, Parkinson's disease, and a host of many different amyloidosis diseases. Protein aggregation and misfolding reactions are also the bane of protein production and impede pharmaceutical drug development. Understanding the fundamental in vivo factors that control the kinetics of protein misfolding is the crucial aspect involved in developing procedures and strategies to avoid this deleterious side reaction. Elegant in vivo work of Nol-len et al. has shown that the elements controlling protein homeostasis such as protein synthesis, energy production in the cell, chaperone-dependent protein folding, protein transport, and protein degradation collectively control intracellular protein aggregation.