1Biomedical Neuroscience Institute (BNI), Faculty of Medicine, Chile; 2Center for Geroscience, Brain Health and Metabolism (GERO), Chile; 3The Buck Institute for Research on Aging, USA.
Claudio Hetz was originally trained as Molecular Biotechnology Engineer at the University of Chile and performed a Ph.D in Biomedical Sciences at Serono Pharmaceutical Research Institute, Switzerland. Then he did his postdoctoral training at Harvard University. He joined the University of Chile during 2007 and is currently Full Professor at the Institute of Biomedical Sciences of the University of Chile and Director of the Biomedical Neuroscience Institute (BNI) and Adjunct Professor at the Buck Institute in California. His research focused on understanding the molecular basis of protein folding stress, its relationship to pathological conditions affecting the nervous system, the generation of new animal models, and the development of prototypic strategies to prevent neuronal damage. He has received important award including the TWAS-ROLAC Young Scientist Prize as outstanding young scientist in Latin America, was finalist in the Eppendorf and Science Award in Neurobiology, and was awarded with the Cell Biology Society and Bios-Chile prize as the best young scientist of Chile.
Most neurodegenerative diseases share common molecular signatures including the accumulation of misfolded proteins in the brain. Alteration in the buffering capacity of the proteostasis network is proposed as one of the triggering steps leading to abnormal protein aggregation. The endoplasmic reticulum (ER) is a major node of the proteostasis network altered in brain diseases and during aging. ER stress triggers a signaling reaction known as the unfolded protein response (UPR), which aims restoring proteostasis through the induction of adaptive programs or the activation of cell death programs when damage is chronic and cannot be repaired. In this talk we discuss our efforts to assess the significance of the UPR to brain aging and disease, in addition to develop gene therapy strategies to alleviate ER stress. A new concept is emerging where depending on specific UPR component targeted and the disease model tested distinct and even opposite effects can be observed on the pathology.
Host: Jaime Espinoza Ruiz
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