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Endoplasmic reticulum (ER) chaperones are multifunctional proteins involved in a variety of biological processes such as protein folding and quality control in the ER, unfolded protein response, MHC class I antigen processing, as well as other important functions these proteins play outside of the ER. The role of ER chaperones in various human diseases seems to be especially important. In recent years there are growing amount of data demonstrating involvement of particular ER chaperones in many pathological processes. These recent findings suggest possible application of ER chaperones in therapeutic trials and development of new pharmaceuticals. There is also a wide field for other applications using chaperones in fundamental and applied studies. The recombinant protein expression technologies should be considered for efficient and safe production of these proteins.