Post ER quality control : a role for UDP-glucose : glycoprotein glucosyl transferase and p97
Fregno, IlariaInstitute for Research in Biomedicine (IRB), Faculty of Biomedical Sciences, Università della Svizzera italiana, Switzerland - Department of Biology, Swiss Federal Institute of Technology, CH-8093 Zurich, Switzerland
Molinari, MaurizioInstitute for Research in Biomedicine (IRB), Faculty of Biomedical Sciences, Università della Svizzera italiana, Switzerland - Ecole Polytechnique Fédérale de Lausanne, School of Life Sciences, CH-1015 Lausanne, Switzerland
22.07.2016
Published in:
Journal of rare diseases research & treatment. - 2016, vol. 1, no. 1, p. 40-42
English
Human proteinopathies are diseases caused by the expression of defective gene products. In some cases, these diseases involve the degradation of mutant but otherwise functional proteins by the quality control system of the secretory pathway. Our recent study identified two proteins that play a role in post-endoplasmic reticulum (ER) quality control and are potential targets for therapeutic treatment of several proteinopathies.