Man

Claudio Andres Hetz Flores

Deputy Director

INSTITUTO DE CIENCIAS BIOMÉDICAS, FACULTAD DE MEDICINA, UNIVERSIDAD DE CHILE

SANTIAGO, Chile

Líneas de Investigación


Neurociencia; Biologia del Desarrollo; Biologia Molecular; Cellular Stress Related to Protein Misfolding; Cell Signaling and Apoptosis; Neurodegeneration

Educación

  •  PhD. In Biomedical Sciences, UNIVERSIDAD DE CHILE. Chile, 2004
  •  Molecular Biotechnology Engineer, UNIVERSIDAD DE CHILE. Chile, 2000
  •  Postdoctoral Research Fellow, HARVARD SCHOOL OF PUBLIC HEALTH. Estados Unidos, 2005

Experiencia Académica

  •   Postdoctoral Research Fellow Other

    DANA-FARBER CANCER INSTITUTE

    Department of Cancer Immunology & AIDS

    Estados Unidos

    2004 - 2005

  •   Postdoctoral Research Fellow Other

    HARVARD SCHOOL OF PUBLIC HEALTH

    Chile

    2005 - 2007

  •   Profesor Asistente/ Investigador Principal Full Time

    UNIVERSIDAD DE CHILE

    Medicina

    Santiago, Chile

    2007 - 2010

  •   Adjunct Professor Other

    HARVARD SCHOOL OF PUBLIC HEALTH

    Estados Unidos

    2009 - 2016

  •   Investigador Asistente Full Time

    UNIVERSIDAD DE CHILE

    Chile

    2015 - A la fecha

  •   Profesor Adjunto Other

    THE BUCK INSTITUTE FOR RESEARCH ON AGING

    Estados Unidos

    2010 - A la fecha

  •   Full Professor and Principal Investigator Full Time

    UNIVERSIDAD DE CHILE

    Medicina

    Chile

    2010 - A la fecha

  •   Director Other

    Santiago, Chile

    2011 - A la fecha

Experiencia Profesional

  •   Deputy Director Full Time

    Biomedical Neuroscience Institute (BNI)

    Santiago, Chile

    2011 - A la fecha

  •   Profesor titular Full Time

    Universidad de Chile

    Santiago, Chile

    2010 - A la fecha

Formación de Capital Humano


(i) Post-doctoral fellows
Dr. Soledad Matus (2007-2012), Dr. Danny Galleguillos (2008-2009), Dr. Rene Vidal (2008-2013); Dr. Andrea Paula (2007-2010; co-advisor); Dr. Karen Castillo (2008-2013), Dr. Gonzalo Encina (2009-2011), Dr. Ute Woehlbier (2010-2014), Dr. Gabriela Mercado (2011-2018), Danilo Bilches (2012-2017), Diego Rojas (2012-2015), Claudia Duran (2013-2017); Pablo Mardones (2013-2017), Paula Huerta (2014-2018); Amado Carreras (2014-2018), Alexis Rivas (2015-2019), Gabriela Martinez (2014-2018), Felipe Cabral (2016-2021), Hery Urra (2016-2018), Vicente Valenzuela (2016-2023), Paulina Falcon (2017-2018), Yannis Gerakis (2017-present), Alfredo Sagredo (2018-2022), Mei-Li Diaz (2019-2022), Philippe Pihan (2018-2024), Younis Hazari (2017-2024), Mateus Milani (2020-2024), Alexis Martinez (2018-2024),

(ii) PhD Thesis
Ph.D. in Biochemistry, University of Chile.
Diego Rojas. (2008-2012) Awarded as Best PhD thesis of Chile, Allende and Connelly Foundation, Chile 2013. Clara Quiroga (2006-2010) (co-advisor). Maria Inés Díaz (2008-2015). Maria Jesus Cerna (2019-2024).

PhD. Biomedical Sciences, University of Chile.
Melissa Calegaro (2007-2012), Mauricio Torres (2007-2013), Gabriela Martinez (2010-2014), Victor Hugo Cornejo (2011-2015), Hery Urra (2011-2014) Awarded as Best PhD thesis of Chile, Chilean Cell Biology Society, Chile 2016 (Federico Leighton Award), Estephanie Dufei (2012-2016), Vicente Valenzuela (2012-2016), Philippe Pihan (2012-2018), Denisse Sepulveda (2013-2018) Awarded as Best PhD thesis of Chile, Chilean Cell Biology Society, Chile 2019. Luis Osorio (2015-2019), Paulina Troncoso (2016-2020), Matias Gonzalez (2016-2020), Celia Limia (2017-2020), Juan Francisco Silvia (2024-Present).

PhD. Medical Sciences, University of Chile.
Lucas Gonzalez (2023-Present), Lene Troncoso (2024-Present).

PhD. Cellular and Molecular Biology, University of Chile.
Pamela Valdés (2008-2013).

Ph.D Pharmacology, University of Chile.
Fernanda Lisbona (2010-2014). Awarded with the Loreal Woman in Science Price (2011) and the RELAB Prize (2012). Rodrigo Perez (2016-2019), Paulina Torres (2017-2021).

PhD in Cellular and Molecular Biology, P. Catholic University of Chile
Alexis Martinez (2012-2016). Co-mentored with Dr Alejandra Alvarez.

PhD in Neurosciences, P. Universidad Católica de Chile.
Maritza Oñate (2013-2018).

PhD in nutrition, University of Chile
María José Hormazábal (2016-2019). Luis Gonzalez (2023-present)

(ii) Master’s students
Master in Biotechnology, Harvard University, USA
Peter Thielen (2010-2011). Awarded with the Dean's Prize for Outstanding ALM Thesis.

Master in Biological Sciences, Faculty of Sciences, University of Chile
Vicente Valenzuela (2009-2010) Awarded as Best Master´s thesis of Chile, Chilean Cell Biology Society, Chile 2012. Andrés Henriquez (2009-2012), Fernanda Lisbona (2007-2008), Maritza Onate (2011-2016) Awarded as Best Master´s thesis of Chile, Chilean Cell Biology Society, Chile 2016, Valentina Castillo (2013-2016), Leslie Bargsted (2013-2016) Pablo Rozas (2014-2018).

Master in Biochemistry, Faculty of Chemistry Sciences, University of Chile.
José Ignacio Astorga (2018-2020)

(iv) Undergraduate Students
Molecular Biotechnology Engineering, Faculty of Science, University of Chile: Sebastian Zamorano (2007-2009), Andrés Henriquez (2007-2009), Vicente Valenzuela (2008-2009), Amparo Zuleta (2010-2011) Catherine Andreu (2011-2012), Leslie Bargted (2012-2013), Valentina Castillo (2012-2014), Martin Sepúlveda (2014-2016).

Biotechnology, Universidad Mayor: Camila Gherardell (2013), Miguel Gonzalez (2014-2015). Biochemistry, University of Chile; Javiera Fernanda Acevedo (2016-2018).

Biotechnology, Universidad Andres Bello. Paloma Moraga (2019-2020). Karina Espinosa (2021-2023).


Difusión y Transferencia


INTELECTUAL PROPERTY Files: PATENTS and LICENCES

Inventors: Claudio Hetz
Title: Reprogramming Proteostasis Network for Sustaining Brain Function During Aging and Extending Health Span.
U.S. Patent Application No.: 63/631,111.
Filed: April 8, 2024 Ref: 94127.10.USP1. Provisional Application Filed.

Inventors: Claudio Hetz and Laurie Glimcher,
Title: MODULATION OF NEURODEGENERATIVE DISEASE BY MODULATING XBP-1 ACTIVITY. APLLICATION NUMBER: US 13/000,921. PCT number: PCT/US2009/048321.
Publication number US 20110142799 A1 (16/6/2011), also refered as WO2010008860A1.

Inventors: Claudio Hetz and Gabriela Martinez.
Title: AAV/XBP1s-ha virus, gene therapy method and use thereof in the optimisation and improvement of learning, memory and cognitive capacities
Chilean application number: 3590-2014(2014). Submitted date 30/12/2014.
International application number: PCT/CL2015/000069. Submitted date 24/12/2015.
International publication number: WO2016106458. Publication date 7/7/2016.
PCT national phases: USA and EPO.
Status: patent pending.

Inventors: Claudio Hetz and Vicente Valenzuela.
Title: Method for genetic treatment using the AAV-XBP1s/GFP virus and use thereof in the prevention and treatment of amyotrophic lateral sclerosis.
Chilean application number: 201503024 (2015). Submitted date 9/10/2015.
International application number: PCT/CL2016/000056. Submitted date 30/09/2016.
International publication number: WO2017059554A1. Publication date 13/04/2017
PCT national phases: USA, EPO and Australia.
Publication number CY1125031T1.

Inventors: Claudio Hetz and Rene Vidal.
Title: AAV/UPR-plus virus, UPR-plus fusion protein, genetic treatment method and use thereof in the treatment of neurodegenerative diseases, such as parkinson's disease and huntington's disease.
Chilean application number: 3242-2015 (2015). Submitted date 4/11/2015.
International application number: PCT/CL2016/000070. Submitted date 4/11/2016.
International publication number: PCT WO2017075729A1. Publication date 11/05/2017.
PCT national phases: Australia, EPO and Japan.
Publication number US11795476B2.


Inventors: Claudio Hetz and Paula Huerta.
Title: AAV-IGF2 as a genetic method to treat protein misfolding disorders such as Huntington´s disease.
Chilean application number: 20160322(2016). Submitted date 21/12/2016.
International application number: PCT/CL2017/000040. Submitted date 21/12/2017.
International publication number: WO2018112672A. Publication date 28/06/2018.
Status: patent pending.

Inventors: Claudio Hetz, Alexis, Carla del Porte, Marcelo Kogan and Daniela Jara.
Title: Use of leaf extracts from Ugni Molinae for the treatment of protein misfolding disorders.
Chilean application number: 3359-2017(2017). Submitted date 26/12/2017.
Status: patent pending.

Inventors: Claudio Hetz, Felipe Cabral.
Title: Treatment of aging or age-related disorders using XBP1.
Provisional application for patent at USPTO, application number 62800229. Submitted 01/02/2019.
Status: patent pending.

Inventors: Claudio Hetz, Felipe Cabral, Gabriela Martinez, Vicente Valenzuela, Rene Vidal.
Title: liscencing of the use of AAV-XBP1 and UPRplus for the treatment of ALS and parkinson´s disease. Licensed to Handl Therapeutics to develop clinical trials.


Premios y Distinciones

  •   Santander Bank Award

    Banco Santander

    Chile, 2016

    For outstanding contributions to University research on the area of neurological diseases

  •   KIA International Award

    Iranian Research Organization for Science and Technology

    Irán, 2015

    First place, for invaluable achievements and contributions to the development of science and technology. In addition, this price was accompanied by an UNESCO special awar

  •   Target Validation Award

    Michael J Fox Foundation for Parkinson Research

    Estados Unidos, 2014

    Para desarrollar terapia genica para el mal de Parkinson.

  •   The Eppendorf & Science Prize for Neurobiology.

    Science

    Chile, 2008

    Selected finalist by the editors of Science magazine as outstanding young scientist.

  •   Highly Ranked Scholars™

    Scholar GPS

    Estados Unidos, 2024

    Selected in the top 0.05% of Highly Ranked Scholars™ for an exceptional performance in various fields, disciplines, and specialties by Scholar GPS™.

  •   Most cited scientist in the world

    Clarivate (Web of Science)

    Chile, 2023

    Awarded by Clarivate (Web of Science) as a “Highly Cited Scientist”, among the TOP 1% most cited scientist in the world

  •   Most cited scientist in Chile

    Research.com

    Chile, 2023

    Selected by Research.com as one of the most cited scientist in Chile in the field.

  •   Highly Cited Scientist

    Clarivate (Web of Science)

    Chile, 2022

    Awarded by Clarivate (Web of Science) as a “Highly Cited Scientist”, among the TOP 1% most cited scientist in the world: https://www.webofscience.com/wos/author/record/2418833

  •   Most cited scientist in Chile

    Research.com

    Chile, 2022

    Selected by Research.com as the most cited scientist in Chile in the field.

  •   Top 2% of most cited scientists

    Elsevier and Stanford

    Chile, 2022

    Selected in the top 2% of most cited scientists by a study performed by Elsevier and Stanford:

  •   WSIS Prize 2022 Champion

    WSIS Prize 2022 Champion

    Chile, 2022

    The project was among the top five most voted in your category.

  •   Highly Cited Scientist

    Clarivate (Web of Science)

    Chile, 2022

    Awarded by Clarivate (Web of Science) as a “Highly Cited Scientist”, among the TOP 1% most cited scientist in the world.


 

Article (94)

Immunohistochemical characterisation of the adult Nothobranchius furzeri intestine
IRE1α Mediates the Hypertrophic Growth of Cardiomyocytes Through Facilitating the Formation of Initiation Complex to Promote the Translation of TOP-Motif Transcripts
c-Abl Phosphorylates MFN2 to Regulate Mitochondrial Morphology in Cells under Endoplasmic Reticulum and Oxidative Stress, Impacting Cell Survival and Neurodegeneration
The ER stress sensor IRE1 interacts with STIM1 to promote store-operated calcium entry, T cell activation, and muscular differentiation
The unfolded protein response transcription factor XBP1s ameliorates Alzheimer's disease by improving synaptic function and proteostasis
ER-mitochondrial contact sites: Balancing energy and protein homeostasis
Mutant PDIA3 causes neurodevelopmental impairment by disturbing endoplasmic reticulum proteostasis.
Mutation in protein disulfide isomerase A3 causes neurodevelopmental defects by disturbing endoplasmic reticulum proteostasis
Unfolded protein response IRE1/XBP1 signaling is required for healthy mammalian brain aging
Adapting the proteostasis capacity to sustain brain healthspan
DEF8 and Autophagy-Associated Genes are Altered in Mild Cognitive Impairment, Probable Alzheimer’s Disease Patients and a Transgenic Model of the Disease
Endoplasmic reticulum stress and unfolded protein response in cardiovascular diseases
Enforced dimerization between XBP1s and ATF6f enhances the protective effects of the UPR in models of neurodegeneration
Protein disulfide isomerase ERp57 protects early muscle denervation in experimental ALS
Acute Pannexin 1 Blockade Mitigates Early Synaptic Plasticity Defects in a Mouse Model of Alzheimer’s Disease
Caveolin-1 suppresses tumor formation through the inhibition of the unfolded protein response
Genotoxic stress triggers the activation of IRE1 alpha-dependent RNA decay to modulate the DNA damage response
Inflammation-associated suppression of metabolic gene networks in acute and chronic liver disease
Insulin-like growth factor 2 (IGF2) protects against Huntington's disease through the extracellular disposal of protein aggregates
Mastering organismal aging through the endoplasmic reticulum proteostasis network
Mechanisms, regulation and functions of the unfolded protein response
The necroptosis machinery mediates axonal degeneration in a model of Parkinson disease
The UPRosome-decoding novel biological outputs of IRE1 alpha function
When Endoplasmic Reticulum Proteostasis Meets the DNA Damage Response
beta-catenin aggregation in models of ALS motor neurons: GSK3 beta inhibition effect and neuronal differentiation
Emerging Roles of the Endoplasmic Reticulum Associated Unfolded Protein Response in Cancer Cell Migration and Invasion
Network approach identifies Pacer as an autophagy protein involved in ALS pathogenesis
Non-canonical function of IRE1 alpha determines mitochondria-associated endoplasmic reticulum composition to control calcium transfer and bioenergetics
The p75(NTR) neurotrophin receptor is required to organize the mature neuromuscular synapse by regulating synaptic vesicle availability
Endoplasmic reticulum stress leads to accumulation of wild-type SOD1 aggregates associated with sporadic amyotrophic lateral sclerosis
Interactome Screening Identifies the ER Luminal Chaperone Hsp47 as a Regulator of the Unfolded Protein Response Transducer IRE1 alpha
IRE1 alpha governs cytoskeleton remodelling and cell migration through a direct interaction with filamin A
Unraveling the role of motoneuron autophagy in ALS
IRE1 signaling exacerbates Alzheimer's disease pathogenesis
Activation of the unfolded protein response promotes axonal regeneration after peripheral nerve injury
ALS-linked protein disulfide isomerase variants cause motor dysfunction
Mystery solved: Trehalose kickstarts autophagy by blocking glucose transport
NF kappa B is a central regulator of protein quality control in response to protein aggregation stresses via autophagy modulation
Regulation of Memory Formation by the Transcription Factor XBP1
ER proteostasis disturbances in Parkinson's disease: novel insights
ER stress signaling and neurodegeneration: At the intersection between Alzheimer's disease and Prion-related disorders
Functional Role of the Disulfide Isomerase ERp57 in Axonal Regeneration
Gene therapy in Parkinson's disease: targeting the endplasmic reticulum proteostasis network
Identification of rare protein disulfide isomerase gene variants in amyotrophic lateral sclerosis patients
PERK regulated miR-424(322)-503 cluster fine-tunes activation of IRE1 and ATF6 during Unfolded Protein Response
The Protein-disulfide Isomerase ERp57 Regulates the Steady-state Levels of the Prion Protein
A new method to measure autophagy flux in the nervous system
Autophagy meets fused in sarcoma-positive stress granules
Cellular Mechanisms of Endoplasmic Reticulum Stress Signaling in Health and Disease. 1. An overview
Control of dopaminergic neuron survival by the unfolded protein response transcription factor XBP1
Interplay Between the Oxidoreductase PDIA6 and microRNA-322 Controls the Response to Disrupted Endoplasmic Reticulum Calcium Homeostasis
Modeling UPR adaptive responses: Understanding the mechanisms that determine cell fate under endoplasmic reticulum (ER) stress had been hampered by the lack of models to study unfolded protein response (UPR) adaptive phases. The development of an engineered protein to conditionally induce its misfolding allowed the establishment of a resolvable ER stress condition
Pathogenic role of BECN1/Beclin 1 in the development of amyotrophic lateral sclerosis
The transcription factor CHOP, a central component of the transcriptional regulatory network induced upon CCl4 intoxication in mouse liver, is not a critical mediator of hepatotoxicity
A failure in energy metabolism and antioxidant uptake precede symptoms of Huntington's disease in mice
Bax Inhibitor-1-mediated Ca2+ leak is decreased by cytosolic acidosis
Functional Contribution of the Transcription Factor ATF4 to the Pathogenesis of Amyotrophic Lateral Sclerosis
Herp depletion protects from protein aggregation by up-regulating autophagy
Measurement of autophagy flux in the nervous system in vivo
Trehalose delays the progression of amyotrophic lateral sclerosis by enhancing autophagy in motoneurons
A BAX/BAK and Cyclophilin D-Independent Intrinsic Apoptosis Pathway
Activation of the unfolded protein response enhances motor recovery after spinal cord injury
Altered Prion Protein Expression Pattern in CSF as a Biomarker for Creutzfeldt-Jakob Disease
Astrocytic alpha(V)beta(3) Integrin Inhibits Neurite Outgrowth and Promotes Retraction of Neuronal Processes by Clustering Thy-1
BH3-only proteins are part of a regulatory network that control the sustained signalling of the unfolded protein response sensor IRE1 alpha
MV-mediated delivery of the transcription factor XBP1s into the striatum reduces mutant Huntingtin aggregation in a mouse model of Huntington's disease
Perk-dependent repression of miR-106b-25 cluster is required for ER stress-induced apoptosis
Targeting the UPR transcription factor XBP1 protects against Huntington's disease through the regulation of FoxO1 and autophagy
TMBIM3/GRINA is a novel unfolded protein response (UPR) target gene that controls apoptosis through the modulation of ER calcium homeostasis
Amyloid beta-Peptide Oligomers Stimulate RyR-Mediated Ca2+ Release Inducing Mitochondrial Fragmentation in Hippocampal Neurons and Prevent RyR-Mediated Dendritic Spine Remodeling Produced by BDNF
Axonal Degeneration Is Mediated by the Mitochondrial Permeability Transition Pore
BAX inhibitor-1 regulates autophagy by controlling the IRE1 alpha branch of the unfolded protein response
Lack of Activation of the Unfolded Protein Response in Mouse and Cellular Models of Niemann-Pick Type C Disease
Oxidative stress activates the c-Abl/p73 proapoptotic pathway in Niemann-Pick type C neurons
Protein folding stress in neurodegenerative diseases: a glimpse into the ER
Alternative Functions of the BCL-2 Protein Family at the Endoplasmic Reticulum
Amyotrophic Lateral Sclerosis Pathogenesis: A Journey Through the Secretory Pathway
HSP72 Protects Cells from ER Stress-induced Apoptosis via Enhancement of IRE1 alpha-XBP1 Signaling through a Physical Interaction
Prion Protein Misfolding Affects Calcium Homeostasis and Sensitizes Cells to Endoplasmic Reticulum Stress
BAX Inhibitor-1 Is a Negative Regulator of the ER Stress Sensor IRE1 alpha
XBP-1 deficiency in the nervous system protects against amyotrophic lateral sclerosis by increasing autophagy
XBP-1 deficiency in the nervous system reveals a homeostatic switch to activate autophagy
Unfolded protein response transcription factor XBP-1 does not influence prion replication or pathogenesis
Perturbation of endoplasmic reticulum homeostasis facilitates prion replication
Prion Pathogenesis is Independent of Caspase-12
Bax channel inhibitors prevent mitochondrion-mediated apoptosis and protect neurons in a model of global brain ischemia
In vitro generation of infectious scrapie prions
The disulfide isomerase Grp58 is a protective factor against prion neurotoxicity
Prion replication alters the distribution of synaptophysin and caveolin 1 in neuronal lipid rafts
Caspase-12 and endoplasmic reticulum stress mediate neurotoxicity of pathological prion protein
Microcin E492, a channel-forming bacteriocin from Klebsiella pneumoniae, induces apoptosis in some human cell lines
Nonselective cation channels as effecters of free radical-induced rat liver cell necrosis
Structure, organization and characterization of the gene cluster involved in the production of microcin E492, a channel-forming bacteriocin
Control of lysosomal-mediated cell death by the pH-dependent calcium channel RECS1

Abstract (13)

Regulation of the endolysosomal pathway by beclin-1 and its role in the proteolytic processing of the amyloid precursor protein
Bci is a new component of the autophagy pathway
Depletion of Beclin-1 increases the delivery of cathepsin D and EGF receptor to lysosomes.
Endoplasmic reticulum stress responses to disrupted endoplasmic reticulum ca2+homeostasis
Functional study of the IRE1 interactome: fine tunning of UPR signaling
Protein disulfide isomerase gene variants are associated with amyotrophic lateral sclerosis
Depletion of Beclin-1 reduces the levels of carboxy terminal fragment beta of the amyloid precursor protein by a mechanism dependent on autophagosome function
Monocarboxylate are not important to support synaptic activity in Huntington's disease
Perk-dependent repression of miR-106b-25 cluster is required for ER stress induced apoptosis
Traffic of ascorbic acid transporter SVCT2 is impaired in Huntington's disease
Protein folding stress in neurodegenerative disease: an interplay between ER stress and autophagy
Correlation of lactate dehydrogenase activity and 14-3-3 protein in cerebrospinal fluid from patients with Creutzfeldt-Jakob disease
Caspase-dependent necrosis-like cell death downstream of the Fas receptor is associated with lipid scrambling and delayed ceramide increase in lymphoid cells

BookSection (1)

Assays to Study IRE1 Activation and Signaling

BookWhole (5)

Emerging roles of the unfolded protein response (UPR) in the nervous system: A link with adaptive behavior to environmental stress?
Dual role of autophagy in neurodegenerative diseases: The case of amyotrophic lateral sclerosis
When the Good Turns Bad: Challenges in the Targeting of Autophagy in Neurodegenerative Diseases
Protein misfolding disorders: A trip into the ER
Targeting endoplasmic reticulum stress pathways to treat neurological disorders associated with protein misfolding

CaseReport (1)

Desafíos en el diagnóstico de enfermedad de Creutzfeldt-Jakob: Caso clínico

Editorial (3)

Meet our associate editor
BCL-2 protein family. Essential regulators of cell death. Preface.
Preface

EditorialMaterial (26)

ER stress links aging to sporadic ALS
Targeting endoplasmic reticulum acetylation to restore proteostasis in Alzheimer's disease
Peroxisomes Get Loud: A Redox Antidote to Hearing Loss
RNA metabolism: putting the brake on the UPR
Stressing out the ER in aminoglycoside-induced hearing loss
Theme Series - UPR in cancer
A Novel ER Stress-Independent Function of the UPR in Angiogenesis
Cell-Nonautonomous Control of the UPR: Mastering Energy Homeostasis
Common Ground: Stem Cell Approaches Find Shared Pathways Underlying ALS
Memory loss in Alzheimer's disease: are the alterations in the UPR network involved in the cognitive impairment?
RESETing ER proteostasis: selective stress pathway hidden in the secretory route
Proteostasis Impairment: At the Intersection between Alzheimer's Disease and Diabetes
Signaling in Cell Death, Survival, Proliferation and Degeneration
THE BIOLOGICAL MEANING OF THE UPR
Unspliced XBP1 controls autophagy through FoxO1
Autophagy impairment: a crossroad between neurodegeneration and tauopathies
Cell-nonautonomous control of the UPR
Crosstalk between the UPR and autophagy pathway contributes to handling cellular stress in neurodegenerative disease
Hormesis Protecting neurons against cellular stress in Parkinson disease
The ER in 4D: a novel stress pathway controlling endoplasmic reticulum membrane remodeling
Protein homeostasis networks in physiology and disease
Targeting autophagy in ALS A complex mission
The UPR as a survival factor of cancer cells: More than folding proteins?
Turning off the unfolded protein response An interplay between the apoptosis machinery and ER stress signaling
Apoptosis, necrosis and autophagy: From mechanisms to biomedical applications
Identification by transcription profiling in human colon carcinoma cells of genes regulated by tumor suppressor Caveolin-1

Generic (1)

Saved by the Matrix: UPR Independent Survival under ER Stress

Letter (1)

The proapoptotic BCL-2 family member BIM mediates motoneuron loss in a model of amyotrophic lateral sclerosis

NewsItem (1)

PROTEIN HOMEOSTASIS Modeling UPR adaptive responses

Note (2)

ERp57 as a novel cellular factor controlling prion protein biosynthesis: Therapeutic potential of protein disulfide isomerases
ERp57 in neurodegeneration and regeneration

Proyecto (40)

Defining thesignificance of the Unfolded Protein Response (UPR) as a therapeutic target to treat liver fibrosis
Dissecting the cell-type specific contribution of the UPR to the synucleinopathy in Parkinson’s disease
Geroscience Center for Brain Health and Metabolism
Improving proteostasis to reduce the adverse effects of stress in the elderly.
Protective role of MyDGF against ER stress.
Research proposal to BioSynaptica: ALS preclinical study to evaluate therapeutic effects of an engineered form of recombinant human erythropoietin at the Biomedical Neuroscience
Role of the unfolded protein response (UPR) in the progression of brain cancer. 

Terapia génica para tratar la esclerosis lateral amiotrófica (ELA) mediante una mejora proteostática
Coping with environmental stress: at the interphase between neuronal proteostasis and brain physiology
Uncovering the Contribution of Cell-Type-Specific ER Proteostasis Networks to PD Pathogenesis
Defining the role of ER stress signaling in C9orf7-mediated ALS. 

Prueba de concepto preclínica: Una terapia génica para tratar ELA-DFT
UPRplus: A novel gene therapy to treat Parkinson´s disease
Gene therapy to restore ER proteostasis imbalance in Parkinson disease
Proteostaser-1: a gene therapy to reduce neurodegeneration and improve cognitive function in Alzheimers disease
ER stress in post-traumatic distress order
Novel Roles of the unfolded protein response (UPR) in cancer
Disrupted proteostasis as a shared mechanism of cognitive decline in normal aging and Alzheimer´s disease
Interfere to Suppress or Promote IRE1 (INSPIRED) RISE Program
Neuromorphic Inspired Science to Maximize Big Data Dynamic Problem Solving for Future intelligence, Surveillance, and Reconnaissance Operations.
Targeting the endoplasmic reticulum (ER) stress sensor IRE1 to treat SOD1-mediated ALS
A novel function of the Unfolded Protein Response (UPR) in learning and memory
Role of IRE1 interactome in cell migration and cancer.
The UPRosome: novel regulatory checkpoints and physiological outputs.
GENE THERAPY STRATEGY TO TARGET THE UNFOLDED PROTEIN RESPONSE IN ALS.
A role of ER stress in wild-type SOD1 misfolding: A model for sALS
Cellomics: An automatized platform for high throughput microscopy with biotechnological potential.
Defining the function of protein Disulfide Isomerases in ALS
Targeting the unfolded protein response (UPR) transcription factor XBP1 to alleviate neurodegeneration triggered by alpha-synuclein
UPRplus: A novel gene therapy to treat Parkinson´s disease.
Biomedical Neuroscience Institute (BNI).
Defining the contribution of the disulfide isomerase ERp57 to the pathogenesis of Prion-related disorders
Millennium Nucleus for Neural Morphogenesis (NEMO).
Role of the Unfolded Protein Response (UPR) in tumor suppression by caveolin-1.
The tumor suppressor activity of Caveolin 1 is associated with an inhibition of the UPR
A role of the UPR in Parkinson's disease
Regulation of neuronal apoptosis by Abl in Alzheimer's disease.
Targeting Autophagy for the removal of mutant SOD1 in ALS.
Targeting Autophagy for the treatment of Protein Conformational Disorders affecting the nervous system.
Center for Molecular Studies of the Cell (CEMC)

Review (34)

Autophagy in hepatic adaptation to stress
Pharmacological targeting of the unfolded protein response for disease intervention
Small Molecules to Improve ER Proteostasis in Disease
Targeting of the unfolded protein response (UPR) as therapy for Parkinson's disease
The UFMylation System in Proteostasis and Beyond
ER stress and the unfolded protein response in neurodegeneration
Endoplasmic Reticulum Stress and the Hallmarks of Cancer
Control of systemic proteostasis by the nervous system
ER proteostasis addiction in cancer biology: Novel concepts
Proteostasis control by the unfolded protein response
Targeting the unfolded protein response for disease intervention
TMBIM protein family: ancestral regulators of cell death
Addicted to secrete - novel concepts and targets in cancer therapy
Disturbance of endoplasmic reticulum proteostasis in neurodegenerative diseases
Targeting autophagy in neurodegenerative diseases
An ERcentric view of Parkinson's disease
ER dysfunction and protein folding stress in ALS
Role of the Unfolded Protein Response in Organ Physiology: Lessons from Mouse Models
Targeting the unfolded protein response in disease
The unfolded protein response in Alzheimer's disease
When ER stress reaches a dead end
Guidelines for the use and interpretation of assays for monitoring autophagy
Protein disulfide isomerases in neurodegeneration: From disease mechanisms to biomedical applications
The unfolded protein response: controlling cell fate decisions under ER stress and beyond
Converging Pathways in the Occurrence of Endoplasmic Reticulum (ER) Stress in Huntington's Disease
Integrating stress signals at the endoplasmic reticulum: The BCL-2 protein family rheostat
Modulating stress responses by the UPRosome: A matter of life and death
THE UNFOLDED PROTEIN RESPONSE: INTEGRATING STRESS SIGNALS THROUGH THE STRESS SENSOR IRE1 alpha
Fine-Tuning of the Unfolded Protein Response: Assembling the IRE1 alpha Interactome
The daily job of night killers: alternative roles of the BCL-2 family in organelle physiology
The stress rheostat: An interplay between the unfolded protein response (UPR) and autophagy in neurodegeneration
Beyond apoptosis: Nonapoptotic cell death in physiology and disease
Molecular mechanisms of neurotoxicity of pathological prion protein
Protein misfolding and disease: the case of prion disorders
1
Francisca Bronfman

Full Professor

Instituto de Ciencias Biomédicas

Universidad Andrés Bello

Santiago, Chile

1
Marcelo Baeza

Profesor Titular

Ciencias Ecologicas

Universidad de Chile

Santiago, Chile

3
Alvaro Glavic

Associate Professor

Departamento de Biología

Universidad de Chile

Santiago, Chile

3
Alejandra Alvarez

Full professor

Cell and Molecular Biology

PONTIFICIA UNIVERSIDAD CATÓLICA DE CHILE, FACULTAD DE CIENCIAS BIOLÓGICAS

Santiago, Chile

1
Maria Bono

Profesor titular

Biologia

UNIVERSIDAD DE CHILE

Santiago, Chile

4
SERGIO LAVANDERO

Director

Advanced Center for Chronic Diseases (ACCDiS)

UNIVERSIDAD DE CHILE, FAC CIENCIAS QUÍMICAS Y FARMACÉUTICAS

Santiago, Chile

1
Mario Chiong

Profesor

Departamento de Bioquímica y Biología Molecular

Universidad de Chile

Santiago, Chile

2
Andrew Quest

Full Professor

Biologia Celular y Molecular

FACULTAD DE MEDICINA, UNIVERSIDAD DE CHILE

Santiago, Chile

1
Cecilia Vergara

Profesor Asociado

Biología

DEPARTAMENTO BIOLOGIA, FACULTAD DE CIENCIAS, U. DE CHILE

Santiago, Chile

1
Marco Fuenzalida

Full Professor

Physiology

UNIVERSIDAD DE VALPARAÍSO

Valparaíso, Chile

1
Erwin Strahsburger

Instructor Professor

Universidad Arturo Prat

Iquique, Chile

3
Andres Stutzin

Full Professor

ICBM

FACULTAD DE MEDICINA UNIVERSIDAD DE CHILE

Santiago, Chile

222
Claudio Hetz

Deputy Director

INSTITUTO DE CIENCIAS BIOMÉDICAS, FACULTAD DE MEDICINA, UNIVERSIDAD DE CHILE

SANTIAGO, Chile

3
Alicia Colombo

Profesora Asociada/Director Científico Biobanco de Tejidos y Fluidos de la Universidad de Chile

Departamento de Anatomía Patológica

Universidad de Chile

Santiago, Chile

2
Andrés Couve

Profesor Titular

Neurociencia

FACULTAD DE MEDICINA, UNIVERSIDAD DE CHILE

Santiago, Chile

1
Agustin Martinez

Profesor Titular

Instituto de Neurociencia

UNIVERSIDAD DE VALPARAÍSO, CENTRO INTERDISCIPLINARIO DE NEUROCIENCIAS DE VALPARAÍSO

Valparaíso, Chile

3
GONZALO MARDONES

Associate Professor

Medicine

Universidad San Sebastián

Valdivia, Chile

2
Rosalba Lagos

Professor

Biología

UNIVERSIDAD DE CHILE, FACULTAD DE CIENCIAS, DEPARTAMENTO DE BIOLOGÍA

Santiago, Chile

6
Ricardo Armisen

Associate Professor

Centro de Genética y Genómica

Universidad del Desarrollo

Santiago, Chile

3
JUAN PABLO HENRIQUEZ

Associate Professor

Cell Biology

UNIVERSIDAD DE CONCEPCIÓN, FACULTAD DE CIENCIAS BIOLÓGICAS, DEPARTAMENTO DE BIOLOGÍA CELULAR

Concepción, Chile

1
Pablo Henny

Profesor Asociado

Anatomía Normal

Pontificia Universidad Católica de Chile

Santiago, Chile

2
Alexis Rivas

Investigador Postdoctoral

Instituto de Neurociencias Biomédicas

Universidad de Chile

Santiago, Chile

2
Luis Barros

Principal Research

Laboratorio Biología Molecular

CENTRO DE ESTUDIOS CIENTÍFICOS

Valdivia, Chile

1
Nelson Osses

Associated Professor

Institute of Chemistry

PONTIFICIA UNIVERSIDAD CATÓLICA DE VALPARAÍSO

Valparaíso, Chile

13
René Vidal

Profesor Asociado

Universidad Mayor

Santiago, Chile

1
Tatiana Adasme

Coordinadora de Estudios

Oficina de Apoyo a la Investigación Clínica

Universidad Chile

Santiago, Chile

2
Daniel Henriquez

Director Ciencia Transversal

Fundación Centro MERI

Santiago, Chile

8
Karen Castillo

Académica Investigadora

,Centro de Investigación de Estudios Avanzados del Maule (CIEAM)

Universidad Catolica del Maule

Talca, Chile

1
Rodrigo Troncoso

Assistant professor

Universidad de Chile, Instituto de Nutrición y Tecnología de los Alimentos

Santiago de Chile, Chile

2
Brigitte van Zundert

Full Professor

UNIVERSIDAD ANDRÉS BELLO, CENTRO DE INVESTIGACIONES BIOMÉDICAS

Santiago, Chile

3
Vicente Torres

Associate Professor

Institute for Research in Dental Sciences

FACULTAD DE ODONTOLOGÍA - UNIVERSIDAD DE CHILE

Santiago, Chile

1
Lisette Leyton

Full Professor

Cell and Molecular Biology

FACULTAD DE MEDICINA UNIVERSIDAD DE CHILE

Santiago, Chile

1
Hans Richter

Profesor Titular

Facultad de Medicina

Universidad Austral de Chile

Valdivia, Chile

4
Adrian Palacios

Profesor

Instituto de Neurosciencia

UNIVERSIDAD DE VALPARAISO

Valparaiso, Chile

1
Nibaldo Inestrosa

Full Professor

PONTIFICIA UNIVERSIDAD CATÓLICA DE CHILE

Santiago, Chile

1
Ariel Castro

Full Professor

Department of Biochemistry and Molecular Biology

UNIVERSIDAD DE CONCEPCION

concepciòn, Chile

1
Patricio Manque

VICE PRESIDENT FOR RESEARCH AND FULL PROFESSOR

UNIVERSIDAD MAYOR

Santiago, Chile

1
Miguel Concha

Full Professor

Anatomy and Developmental Biology

UNIVERSIDAD DE CHILE

Santiago, Chile

6
Diego Rojas

Assistant Professor

Center for Integrative Biology

Universidad Mayor

Santiago, Chile

1
Clara Quiroga

Profesor Asistente Adjunto

División de Enfermedades Cardiovasculares

Pontificia Universidad Católica de Chile

Santiago, Chile

8
Danilo Bilches

Staff Scientist

Instituto de Neurociencia Biomedica

Santiago, Chile

1
Fabián Jaña

Profesor Asistente

Departamento de Ciencias de la Salud

Universidad de Aysén

Coyhaique, Chile

1
Claudio Cabello

Full Professor

Ciencias Biologicas

UNIVERSIDAD ANDRES BELLO

Santiago, Chile

1
Gonzalo Encina

Investigador

Instituto de Ciencias e Innovación en Medicina

Universidad del Desarrollo

Santiago, Chile

1
MIGUEL PEREZ

Associate Professor

Universidad Vina del Mar

Vina del Mar, Chile

1
Arlek Gonzalez

INVESTIGADOR POST-DOCTORAL

CENTRO INTERDISCIPLINARIO DE NEUROCIENCIA, UNIVERSIDAD DE VALPARAISO

Valparaiso, Chile

5
Silvana Zanlungo

Full Professor

Gastroenterology

Pontificia Universidad Católica de Chile

Santiago, Chile

2
Jimena Sierralta

Full Professor

Neuroscience

Faculty of Medicine, Universidad de Chile

Santiago, Chile

1
Sebastian Brauchi

Associate Professor

Physiology

Universidad Austral de Chile

Valdivia, Chile

1
M. Cecilia Hidalgo

Professor

ICBM

Universidad de Chile

Santiago, Chile

1
Patricio Godoy

Group leader

System sToxicology

Leibniz Research Centre for Working Environment and Human Factors at the TU Dortmund

Dortmund, Alemania

2
Julio Cardenas

Associated Professor

Center for Integrative Biology

Universidad Mayor de Chile

Santiago, Chile

1
Andrea Paula-Lima

Assistant Professor

Institute for research in Dental Sciences

Universidad de Chile

Santiago, Chile

5
Jose Matamala

Assistant Professor of Neurology

Department of Neurological Science

University of Chile

Santiago, Chile

6
Christian Gonzalez

Full Professor

Biologia

Universidad de Chile

Santiago, Chile

1
Fabiola Osorio

Associate Professor

Programa Disciplinario de Inmunologia

Universidad de Chile

Santiago, Chile

16
Felipe Court

Full Professor and Director

Center for Integrative Biology

Universidad Mayor

Huechuraba, Chile

1
Roberto Bravo

Assistant Professor

Institute of Nutrition and Food Technology (INTA), Universidad de Chile

Santiago, Chile

6
Víctor Cornejo

Profesor Asistente

Facultad de Ciencias Biológicas

Pontificia Universidad Católica de Chile

Santiago, Chile

7
Hery Urra

Staff scientist

INSTITUTO DE NEUROCIENCIA BIOMEDICA (BNI)

Santiago, Chile

1
Karina Palma

Postdoctorado

Instituto de Neurociencia Biomédica BNI

Universidad de Chile

Santiago, Chile

2
Gonzalo Cancino

Assistant Professor

Faculty of Biological Sciences

Pontificia Universidad Católica de Chile

Santiago, Chile

1
Marcelo Catalan

Profesor Auxiliar

Facultad de Medicina

Universidad Austral de Chile

Valdivia, Chile

5
Pablo Mardones

Research Associate

PONTIFICIA UNIVERSIDAD CATOLICA DE CHILE

Santiago, Chile

1
Carlos Spichiger

Profesor Adjunto

Facultad de Ciencias

UNIVERSIDAD AUSTRAL DE CHILE

Valdivia, Chile

13
Melissa Calegaro

Principal Investigator, head lab

Research Vice Presidency

Universidad Mayor

Santiago, Chile

6
Claudia Duran-Aniotz

Profesor Asociado

Escuela de Psicología

Universidad Adolfo Ibánez

Santiago, Chile

1
Daniela López

Profesora Adjunta

UNIVERSIDAD DE VALPARAISO

Viña de Mar, Chile

1
Washington

Postdoctoral Researcher

Fundación Ciencia y Vida

Santiago, Chile

1
Natalia Salvadores

Profesora Asistente

Centro de Resiliencia, Adaptación y Mitigación

Universidad Mayor de Chile

Temuco, Chile

2
Felipe Beltrán

Gestor Tecnológico

I+D

Consorcio Apícola

Valdivia, Chile

1
Cheril Tapia

Profesor Asistente

Facultad de Medicina y Ciencia

Universidad San Sebastián

Santiago, Chile

2
Valentina Valenzuela

Investigador Asociado

Centro de Biotecnologia

Universidad de Concepción

Concepción, Chile

1
Carol San Martín

Profesora Asistente

Neurología y Neurocirugía

Universidad de Chile

Santiago, Chile

1
Pedro Chana Cuevas

Profesor Titular

Facultad de Ciencias Medicas

Universidad de Santiago

Santiago, Chile

1
Gino Corsini

Director Instituto Ciencias Biomédicas

Instituto de Ciencias Biomédicas

Universidad Autónoma de Chile

Santiago, Chile

3
Catherine Andreu

Estudiante de Doctorado

Escuela de Psicología

Pontificia Universidad Católica de Chile

Santiago, Chile

9
Maria Lisbona

Postdoctorado

Biología

Universidad de Chile

Santiago, Chile

2
Alfredo Sagredo

Investigador Postdoctoral

ICBM

Universidad de Chile

Santiago, Chile

4
Philippe Pihán

Posdoctorado

Fisiología

Universidad de Chile

Santiago, Chile

1
Alejandra Catenaccio

Asistente de Investigación

Universidad Mayor

HUECHURABA, SANTIAGO, Chile

12
Ute Wohlbier

Associate Professor

Center for Integrative Biology

Universidad Mayor de Chile

Santiago, Chile

2
ANDRES KLEIN

Assistant Professor/Director (Head) Center for Genetics and Genomics; Director, PhD Program in Sciences and Innovation in Medicine

Centro de Genética y Genómica

Universidad del Desarrollo-Clínica Alemana

SANTIAGO, Chile

1
Rodrigo Troncoso

Associate Professor

Instituto de Nutrición y Tecnología de los Alimentos

Santiago, Chile

1
Zully Pedrozo

Associated Professor

Physiology and Biophysical

Universidad de Chile

SANTIAGO DE CHILE, Chile

6
Alvaro Ardiles

Full Professor

Medicine

Universidad de Valparaíso

Valparaiso, Chile

20
Maria Matus

Principal Investigator

Fundacion Ciencia & Vida

Santiago, Chile

1
Fernando Bustos

Profesor Asistente

Facultad de Medicina

Universidad Andres Bello

Santiago, Chile

1
Rodrigo Herrera

Group Leader (staff member)

Combinatorial NeuroImaging Core Facility

Leibniz Institute for Neurobiology

Magdeburg, Alemania

1
Claudio Cordova

Investigador asistente

Medicina

Universidad de Valparaíso

Valparaíso, Chile

1
Janina Borgonovo

PhD

Programa de Anatomía y Biología del Desarrollo

Universidad de Chile, Facultad de Medicina

Santiago, Chile

2
Cristina Pinto

Doctor en ciencias biologicas

Centro de Biotecnología

Universidad de Concepción

Concepción, Chile

12
Gabriela Martinez

Profesor Asociado

Filosofía y educación

Universidad Metropolitana de Ciencias de la Educación

santiago, Chile

4
Alexis Martinez

Investigador postdoctoral senior

Facultad de medicina

Instituto de Neurcoeincia Biomedica

Santiago, Chile

4
DENISSE SEPULVEDA

Postdoc

Universidad Mayor de Chile

Santiago, Chile

1
Johanna Ábrigo

Bioquímico

Centro de Bioinformática y Biología Integrativa

Santiago, Chile

2
Jorge Ojeda

Académico investigador

Ciencias Básicas y Morfología

Universidad Católica de la Santisima Concepción

Concepción, Chile

4
Viviana Pérez

Profesor Asistente

Fisiología

Universidad de Concepción

Concepcion, Chile

1
Carlos Rozas

Profesor asistente

Biologia

Universidad de Santiago de Chile

Santiago, Chile

1
Francisco Fuentes

Profesor Asistente

Departamento de Microbiología

Universidad de Concepción

Concepción, Chile

1
Camilo Allende

Docente

Universidad del Desarrollo

Santiago, Chile

1
Carolina Flores

Investigador Postdoctoral

Neurociencias

Universidad de Valparaíso

Valparaíso, Chile

1
Patricia Ojeda

Research Assistant

Facultad de medicina

Universidad de Chile

Santiago, Chile

7
Pablo Rozas

Investigador

SymbiOx

Santiago, Chile

1
Paulina Troncoso

Bioquimica PhD

Laboratorio Clínico Banco de Sangre Anatomía Patológica

Clínica Alemana de Santiago

Santiago, Chile

2
Celia Limia

N/A

N/A

Santiago, Chile

3
Valentina Castillo

Investigadora Postdoctoral

Instituto de Ingeniería Biológica y Médica

Pontificia Universidad Católica de Chile

Santiago, Chile

1
Marisol Cisternas

PHD c

Universidad Mayor de Chile

Santiago, Chile

2
sandra espinoza

asistente de investigacion

Laboratorio Neuroinmunologia

Fundacion Ciencias para la Vida/Universidad San Sebastian

Santiago, Chile

1
Estefania Lopez

Lab Manger /Asistente de investigacion

Neurociencias

Universidad de Chile

Santiago, Chile