Research Topics
Species | Claudio A HetzSummaryAffiliation: Universidad de Chile Country: Chile Publications
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Detail Information
Publications
XBP-1 deficiency in the nervous system reveals a homeostatic switch to activate autophagySoledad Matus
Institute of Biomedical Sciences, the FONDAP Center for Molecular Studies of the Cell CEMC, and Millennium Nucleus for Neural Morphogenesis NEMO, Faculty of Medicine, University of Chile, Santiago, Chile
Autophagy 5:1226-8. 2009..Here, we discuss possible signaling pathways that may connect an impaired UPR with the induction of autophagy...
The unfolded protein response: integrating stress signals through the stress sensor IRE1αClaudio Hetz
Department of Immunology and Infectious Diseases, Harvard School of Public Health, Boston, MA, USA
Physiol Rev 91:1219-43. 2011....
XBP-1 deficiency in the nervous system protects against amyotrophic lateral sclerosis by increasing autophagyClaudio Hetz
Institute of Biomedical Sciences, The FONDAP Center for Molecular Studies of the Cell CEMC and the Millennium Nucleus for Neural Morphogenesis NEMO, University of Chile, Santiago, Chile
Genes Dev 23:2294-306. 2009..Our results reveal a new function of XBP-1 in the control of autophagy and indicate critical cross-talk between these two signaling pathways that can provide protection against neurodegeneration...
Fine-tuning of the unfolded protein response: Assembling the IRE1alpha interactomeClaudio Hetz
The FONDAP Center for Molecular Studies of the Cell, Institute of Biomedical Sciences, University of Chile, Santiago, Chile
Mol Cell 35:551-61. 2009..In this Review, we discuss recent evidence supporting a model where IRE1alpha signaling emerges as a highly regulated process, controlled by the formation of a dynamic scaffold onto which many regulatory components assemble...
Unfolded protein response transcription factor XBP-1 does not influence prion replication or pathogenesisClaudio Hetz
Department of Immunology and Infectious Diseases, Harvard School of Public Health, Boston, MA 02115, USA
Proc Natl Acad Sci U S A 105:757-62. 2008....
The daily job of night killers: alternative roles of the BCL-2 family in organelle physiologyClaudio Hetz
Department of Immunology and Infectious Diseases, Harvard School of Public Health, Boston, MA 02115, USA
Trends Cell Biol 18:38-44. 2008..Here, we describe the emerging concept that BCL-2-related proteins have alternative functions beyond apoptosis to control the essential functions of the cell...
ER stress signaling and the BCL-2 family of proteins: from adaptation to irreversible cellular damageClaudio A Hetz
Department of Cellular and Molecular Biology, Institute of Biomedical Sciences, Faculty of Medicine, University of Chile and the FONDAP Center for Molecular Studies of the Cell CEMC, Santiago, Chile
Antioxid Redox Signal 9:2345-55. 2007..Thus, BCL-2-related proteins are not only the "death gateway" keepers, but they also have alternative functions in essential cellular processes...
Perturbation of endoplasmic reticulum homeostasis facilitates prion replicationClaudio Hetz
Department of Neurology, University of Texas Medical Branch, Galveston, Texas 77555, USA
J Biol Chem 282:12725-33. 2007..Because PrP(Sc) induces ER stress, our data point to a vicious cycle accelerating prion replication, which may explain the rapid progression of the disease...
Proapoptotic BAX and BAK modulate the unfolded protein response by a direct interaction with IRE1alphaClaudio Hetz
Howard Hughes Medical Institute, Dana Farber Cancer Institute, and Harvard Medical School, Boston, MA 02115, USA
Science 312:572-6. 2006..Thus, BAX and BAK function at the ER membrane to activate IRE1alpha signaling and to provide a physical link between members of the core apoptotic pathway and the UPR...
BAX inhibitor-1 is a negative regulator of the ER stress sensor IRE1alphaFernanda Lisbona
Institute of Biomedical Sciences, FONDAP Center for Molecular Studies of the Cell, University of Chile, Santiago, Chile
Mol Cell 33:679-91. 2009..Our results suggest a role for BI-1 in early adaptive responses against ER stress that contrasts with its known downstream function in apoptosis...
Stressing out the ER: a role of the unfolded protein response in prion-related disordersClaudio A Hetz
Harvard School of Public Health, Department of Immunology and Infectious Diseases, Boston, MA 02115, USA
Curr Mol Med 6:37-43. 2006..These findings have applications for developing novel strategies for treatment and early diagnosis of transmissible spongiform encephalopathies and other neurodegenerative diseases...
Molecular mechanisms of neurotoxicity of pathological prion proteinJoaquin Castilla
University of Texas Medical Branch, Galveston, TX 77555, USA
Curr Mol Med 4:397-403. 2004....
The stress rheostat: an interplay between the unfolded protein response (UPR) and autophagy in neurodegenerationSoledad Matus
Program of Cellular and Molecular Biology, Institute of Biomedical Sciences, Faculty of Medicine, University of Chile, Santiago, Chile
Curr Mol Med 8:157-72. 2008..The most recent evidence addressing the role of the UPR and ER stress in neurodegenerative disorders is reviewed here, along with therapeutic strategies to alleviate ER stress in a disease context...
Alternative functions of the BCL-2 protein family at the endoplasmic reticulumDiego Rojas-Rivera
The FONDAP Center for Molecular Studies of the Cell, Institute of Biomedical Sciences, Faculty of Medicine, and Millennium Nucleus for Neural Morphogenesis, University of Chile, Santiago, Chile
Adv Exp Med Biol 687:33-47. 2010..Here we describe the emerging concept that BCL-2 family members are important regulators of essential cellular processes beyond apoptosis...
Cyclophilin D is a component of mitochondrial permeability transition and mediates neuronal cell death after focal cerebral ischemiaAnna C Schinzel
Howard Hughes Medical Institute and Dana Farber Cancer Institute, Harvard Medical School, 44 Binney Street, Boston, MA 02115, USA
Proc Natl Acad Sci U S A 102:12005-10. 2005....
Converging pathways in the occurrence of endoplasmic reticulum (ER) stress in Huntington's diseaseR Vidal
Institute of Biomedical Sciences, FONDAP Center for Molecular Studies of the Cell CEMC, Faculty of Medicine, University of Chile, Santiago, Chile
Curr Mol Med 11:1-12. 2011..Here, we review recent evidence involving ER stress in HD pathogenesis and discuss possible therapeutic strategies to target organelle function in the context of disease...
In vitro generation of infectious scrapie prionsJoaquin Castilla
Department of Neurology, University of Texas Medical Branch, Galveston, Texas 77555, USA
Cell 121:195-206. 2005..These findings demonstrate that prions can be generated in vitro and provide strong evidence in support of the protein-only hypothesis of prion transmission...
Diminishing apoptosis by deletion of Bax or overexpression of Bcl-2 does not protect against infectious prion toxicity in vivoAndrew D Steele
Whitehead Institute for Biomedical Research, Massachusetts Institute of Technology, Cambridge, Massachusetts 02142, USA
J Neurosci 27:13022-7. 2007..These results suggest that Bax and Bcl-2 mediated apoptotic pathways are not the major contributing factor to the clinical or pathological features of infectious prion disease...
Emerging roles of the unfolded protein response signaling in physiology and diseaseClaudio A Hetz
Curr Mol Med 6:1. 2006
