Protein Kvalitetskontrol i det endoplasmatiske reticulum

Protein folding and quality control in the endoplasmic reticulum (ER) are synchronized mechanisms ensuring that only properly folded proteins are intergrated in the plasma membrane or secreted from the cell. These mechanisms act in close collaboration with the molecular machinery involved in retrograde-translocation and degradation of non-native proteins and with the ER-stress activated sinalling system. The common goal of these mechanisms is to prevent ecpression and secretion of misfolded proteins. Protein misfolding can be detrimental to the cell and contributes to the disease mechanism in severeal inherited disorders, e.g. cystic fibrosis, familial hypercholesterolemia and diabetes insipididus. This review outlines the molecular mechanisms in protein quality control occuring in the ER, signalling caused by ER stress, and finally ER associated protein degradation.