Accelerating Discovery

*Publications with affiliation to the University of Chile
#Corresponding author


Martínez G., Vidal R., Mardones P., Serrano F., Ardiles A., Wirth C., Valdés P., Thielen P., Schneider B., Kerr B., Valdés J., Palacios A., Inestrosa N., Glimcher L.H. and Hetz C. (2016). Regulation of memory formation by the transcription factor XBP1. Cell Rep. 14:1382-1394. *#

Woehlbier U, Colombo A, Saaranen M, Pérez V., Ojeda J., Bustos F., Andreu C., Torres M., Valenzuela V., Medinas D., Rozas P., Vidal R., Lopez-Gonzalez R., Salameh J., Fernandez-Collemann S., Muñoz N., Matus S., Armisen R., Sagredo A., Palma K., Irrazabal T., Gonzalez-Perez P., Campero M., Gao F, Henny P., van Zundert B., Ruddock L., Concha M., Henriquez J., Brown R.H. and Hetz C. (2016). ALS-linked Protein Disulfide Isomerase Variants cause motor dysfunction. EMBO J. 35:845-65.*#

Oñate M., Catenaccio A., Martínez G., Armentano D., Parsons G., Kerr B., Hetz C. and Court F. (2016). Activation of the unfolded protein response promotes axonal regeneration after peripheral nerve injury. Scientific Reports. 6:21709. *#

Duran-Aniotz C., Cornejo V., and Hetz C. (2016). Targeting ER acetylation to restore proteostasis in Alzheimer’s disease. Brain. 139:650-652. *# Editorial comment.

Mardones P., Rubinztein D. and Hetz C. (2016). Mystery solved: Trehalose kickstarts autophagy by blocking glucose transport. Science Sigaling. 9(416):fs2. *# Editorial comment.

Nivon M., Fort L., Muller P., Richet E., Simon S., Guey B., Fournier M., Arrigo A.P., Hetz C., Atkin JD, Kretz-Remy C. (2016). NFκB is a central regulator of protein quality control in response to protein aggregation stresses via autophagy modulation. Mol Biol Cell. pii: mbc.E15-12-0835*

Garcia-Huerta P., Troncoso-Escudero P., Jerez C., Hetz C. and Vidal R. (2016). The intersection between growth factors, autophagy and ER stress: a new target to treat neurodegenerative diseases? Brain Res. pii: S0006-8993(16)30128-7. *#

Rozas P., Sepulveda M., Hetz C. and Medinas D. (2016). ERp57 as a novel cellular factor controlling Prion biosynthesis: Therapeutic potential of protein disulfide isomerases. Prion. In press.*#

Klionsky et al (Hetz C) (2016). Guidelines for the use and interpretation of assays for monitoring autophagy(2nd edition). Autophagy. 12:1-222*

Urra H., Dufey E., Avril T., Chevet E. and Hetz C. (2016). ER stress and the hallmarks of cancer. Trends in Cancer. 5:252-262. *#

Rozas P., Bargsted L., Martínez F., Hetz C. and Medinas D.B. (2016). The ER proteostasis network in ALS: explaining the differential motoneuron vulnerability. Neuroscience Letters. pii: S0304-3940(16)30288-9. *#

Hetz C. and Saxena S. (2016). Divergent roles of ER stress in neurodegeneration and brain disorders. Brain Res. In press. *# Editorial comment, special review edition.

Valenzuela V., Martinez G., Duran C., and Hetz C. (2016). Gene therapy to target ER stress in brain diseases. Brain Res. pii: S0006-8993(16)30313-4. *#

Valenzuela V., Oñate V., Hetz C. and Court F. (2016). Injury to the nervous system: a look into the ER. Brain Res. pii: S0006-8993(16)30290-6. *#

Mollereau B, Roussel B.D., Chevet E., Sedru M., Van den Brink D., Bailly-Maitre B., Palladino F., Medinas D.B.,Domingos P.M., Hunot S., Chandran S., Rzechorzek N.M., Birman S., Baron T., Vivien D., Duarte C.B., Ryoo H.D.., Crowder C.M., Urano F., Steller H., Kroemer G., Ciechanover A., Calabrese E., Kaufman R.J. and Hetz C. (2016). Adaptive preconditioning in neurological diseases - therapeutic insights from proteostatic perturbations. Brain Res. pii: S0006-8993(16)30092-0.*

Bargsted L., Hetz C., Matus S. (2016). ERp57 in neurodegeneration and regeneration. Neural Regen Res. 11:232-233. *#

Martinez G., Duran-Aniotz D., Cabral F and Hetz C. (2016). Decay in the buffering capacity of the ER proteostasis network during aging. Frontiers Aging Neuroscience. In press. *#

Sepulveda M., Rozas P., Hetz C., Medinas D.B. (2016). ERp57 as a novel cellular factor controlling prion protein biosynthesis: Therapeutic potential of protein disulfide isomerases. Prion. 10:50-6.*#

Oñate M., Court F., and Hetz C. (2016). Bursting the UPR accelerates axonal regeneration. Neural Regen Res. In press. *#

Ramírez M., Gallardo A., Cornejo A., Vidal A., Ramírez D., Medinas D., Hetz C. , Bustamante G., Pasquali R. (2016). Chalenges in the diagnosis of Creutzfeldt-Jakob: A case report. Rev Med Chilena.In press.*


Mardones P, Hetz C. (2015). Peroxisomes Get Loud: A Redox Antidote to Hearing Loss. Cell. 163:790-1. *

Hetz C., Chevet E., and Oakes S. (2015). Proteostasis control by the unfolded protein response. Nature Cell Biology. 7:829-38. *#

Torres M., Medinas D.B., Matamala J.M., Woehlbier U., Cornejo V.H., Solda T., Andreu C., Rozas P., Matus S., Muñoz N., Vergara C., Cartier L., Soto C., Molinari M., and Hetz C. (2015). The Protein Disulfide Isomerase ERp57 Regulates the Steady-State Levels of the Prion Protein. J Biol Chem. 290:23631-45*#

Castillo V., Oñate M., Woehlbier U., Rozas P., Andreu C., Medinas D., Valdés P., Osorio F., Mercado G., Vidal R.L., Kerr B., Court F.A., andHetz C. (2015).Functional Role of the Disulfide Isomerase ERp57 in Axonal Regeneration. PLoS One. 10:e0136620. *#

Mardones P. Martinez G., and Hetz C. (2015). Control of systemic proteostasis by the nervous system. Trends Cell Biol. 25:1-10*#. (Cover illustration).

Mardones P., DiIllin A. and Hetz C. (2015). Cell-nonautonomous control of the UPR: Mastering energy homeostasis. Cell Metabolism. 20:385-7. Editorial comment. *#

Gupta A., Mosaraf Hossain M., Read D., Hetz C., Samali A. and Gupta S. (2016) PERK regulated miR-424(322)-503 cluster fine-tunes activation of IRE1 and ATF6 during Unfolded Protein Response Sci Rep. 5:18304. *

Rojas-Rivera D. and Hetz C. (2015). TMBIM protein family: ancestral regulators of cell death. Oncogene. 34:269-280.*#

Garcia-Huerta P., Rivas A., and Hetz C. (2015) Stressing out the ER in aminoglycoside-induced hearing loss. Cell Death Dis. 14;6:e1762. Editorial comment. *#

Cardenas-Sureda A. and Hetz C. (2015). RNA metabolism: Putting the brake on the UPR. EMBO Rep. 6:545-6. Editorial comment. *#

Hetz C. and Chevet E. (2015). Theme Series - UPR in cancer. Sem Cancer Biology. 33:1-2. Editorial comment. *#

Chevet E., Hetz C., and Samali A. (2015). Endoplasmic Reticulum Stress-Activated Cell Reprogramming in Oncogenesis. Cancer Discovery. 5:586-97*

Dufey E., Urra H., and Hetz C. (2015). ER proteostasis addiction in cancer biology: Novel concepts . Sem Cancer Biology. 33:40-7*#

Gonzalez-Perez P ., Woehlbier U.,Chian RJ.,Sapp P.,Rouleau GA.,Leblond CS.,Daoud H.,Dion P.A, Landers J.E., Hetz C . , Brown R.H. (2015). Identification of rare protein disulfide isomerase gene variants in amyotrophic lateral sclerosis patients, Gene. 566:158-65. *#

Mercado G., Castillo V., Vidal R. and Hetz C. (2015). ER proteostasis disturbances in Parkinson’s disease: novel insights. Frontiers Aging Neurosci. 7:39. *#

Torres M., Matamala J.M., Duran-Aniotz C., Cornejo V.H., Foley A., Hetz C. (2015) ER stress signaling and neurodegeneration: At the intersection between Alzheimer's disease and Prion-related disorders. Virus Res 207:69-75. *#

Rivas A., Vidal R. and Hetz C. (2015). Pharmacological strategies to target endoplasmic reticulum (ER) stress with chemical and natural compounds. Expert Opinion Therapeutic Targets. 19:1203-1.. *#

Mercado G., Castillo V. and Hetz C. (2015). Mercado G., Gene therapy in Parkinson´s disease: targeting the ER proteostasis network. Neural Regeneration Research. 10:1053-1054. *#


Hetz C. and Mollereau B. (2014)Disturbance of ER proteostasis in neurodegenerative diseases. Nature Rev Neurosci. 15:233-249.*#

Valdés P., Mercado G., Vidal R., Parsons G., Court F., Molina C., Martinez A., Galleguillos D., Armentano D., Schneider B., and Hetz C. (2014). Control of dopaminergic neuron survival by the unfolded protein response transcription factor XBP1. Proc Natl. Acad. Asci USA. 111:6804-9. *#

Groenendyk J., Peng Z., Dudek E., Fan X., Mizianty M., Dufey E., Urra H., Sepulveda D., Rojas-Rivera D., Lim Y., Han Kim D., Baretta K., Srikanth S., Gwack Y., Ahnn J., Kaufman K., Lee S., Hetz C., Kurgan L. and Michalak M. (2014). Interplay Between the Oxidoreductase PDIA6 and microRNA-322 Controls the Response to Disrupted Endoplasmic Reticulum Calcium Homeostasis. Science Signaling 7: 1-14.* (Cover illustration)

Nassif M., Valenzuela V., Rojas-Rivera D., Vidal R., Matus S, Castillo K., Fuentealba Y., Kroemer G., Levine B., and Hetz C. (2014). Pathogenic role of BECN1/Beclin 1 in the development of amyotrophic lateral sclerosis. Autophagy. 10: 1256-1271*#. (Cover illustration).

Urra H. and Hetz C. A novel ER stress-independent function of the UPR in angiogenesis. Mol Cell. 54:542-544. Editorial comment. *#

Medinas D. and Hetz C. (2014). Protein folding stress: Modeling UPR adaptive responses. Nature Chem Biol. 10:879-80. Editorial comment. *#

Vidal R., Matus S., Bargsted L., and Hetz C. (2014). Targeting autophagy in neurodegenerative diseases. Trends Pharmacol Sci. 35: 583-91*# (Cover illustration).

Couve A. and Hetz C. (2014). RESETing ER proteostasis: New stress pathways hidden in the secretory pathway. EMBO J. 33:2444-6. Editorial comment. *#

Matus S., Bosco D., and Hetz C. (2014). Autophagy meets FUS-positive Stress Granules. Neurobiol Dis. 35: 2832-5. Editorial comment. *#

Dufey E., Sepulveda D., Rojas DR., and Hetz C. (2014). ER stress signaling mechanisms: an overview. Am J. Physiol -Cell Physiology. 307: C582-94. *#

Matus S., Medinas D., and Hetz C. Common Ground: Stem Cell Approaches Find Shared Pathways Underlying ALS. Cell Stem Cell. 14: 697-699. Editorial comment. *#

Torres M, Matamala JM, Duran-Aniotz C, Cornejo VH, Foley A, Hetz C. (2014) ER stress signaling and neurodegeneration: At the intersection between Alzheimer's disease and Prion-related disorders. Virus Res. 2014 Dec 31. pii: S0168-1702(14)00523-1. *#

Campos G., Schmidt-Heck W., Ghallab A., Rochlitz K., Pütter L., Medinas D., Hetz C., Widera A, Cadenas C, Begher-Tibbe B., Reif R., Günther G., Sachinidis A., Hengstler J., and Godoy P. (2014). 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. Archives Toxicol.88: 1267-1280.*

Dejeans N., Manié S., Hetz C., Bard F., Hupp T., Agostinis P., Samali A. and Chevet E. (2014). Addicted to secrete – novel concepts and targets in cancer therapy. Trend Mol Med. 20: 242-250.*

Duran-Aniotz C., Martinez G. and Hetz C. (2014). Memory loss in Alzheimer´s disease: Are the alterations in the UPR network involved in the cognitive impairment? Frontiers Aging Neurosci. 6:1-3.*#

Matus S., Valenzuela V. and Hetz C. (2014). A new method to measure of autophagy flux in the nervous system. Autophagy. 10:710–714.*#


Hetz C. (2013). The biological meaning of the UPR.  Nature Rev Mol Cell Biol. 14:404.  Journal Club*#

Hetz C., Chevet E., and Harding H. (2013). Targeting the unfolded protein response in disease. Nature Rev Drug Discovery 12:703-719. *#

Vilches D. and Hetz C. (2013). Proteostasis impairment: A molecular intersection between Alzheimer´s disease and diabetes. Cell Metabolism. 8:771-2. Editorial comment. *#

Acuña A., Esparza M., Kramm C., Beltrán F., Parra A., Cepeda C., Toro C., Vidal R., Hetz C., Concha I., Brauchi S., Levine M. and Castro M. (2013). A failure in energy metabolism and antioxidant uptake precede the onset of Huntington's disease. Nature Communications. 13:2917.*

Castillo K., Valenzuela V., Matus S., Nassif M., Oñate M, Fuentealba Y. Encina G., Irrazabal T., Parsons G., Court F., Schneider B., Armentano D., and Hetz C. (2013)Measurement of autophagy flux in the nervous system in vivo. Cell Death Dis.4:e917. *#

Castillo K., Nassif M., Valenzuela V., Rojas F., Matus S., Mercado G., Court F., van Zundert B., and Hetz C. (2013)Trehalose delays the progression of amyotrophic lateral sclerosis by enhancing autophagy in motoneurons. Autophagy 9:1308-20. *#

Matus S, Lopez E., Valenzuela V., Nasiff M., and Hetz C. (2013)Functional role of the transcription factor ATF4 in the pathogenesis of amyotrophic lateral sclerosis. PloS One 8:e66672.

Mercado G., Valdés P., and Hetz C. (2013). An ERcentric view of Parkinson’s disease. Trends Mol. Med. 19(3):165-75. *#

Hurra H, Dubey E, Lisbona F, Rojas D, and Hetz C. (2013). When ER stress reaches a dead end. Biochim Biophys Acta (BBA) - Molecular Cell Research.S0167-4889(13)00311-X. *#

Kiviluoto S., Luyten T., Schneider L., Lisak D., Rojas-Rivera D., Welkenhuyzen K., Missiaen L., De Smedt H., Parys J., Hetz C., Methner A., Bultynck G. (2013)Bax Inhibitor-1-mediated Ca2+ leak is decreased by cytosolic acidosis. Cell Calcium. 54:186-192. *

Quiroga C., Gatica D., Paredes F., Bravo R., Troncoso R:, Pedrozo Z., Rodriguez A., Vicencio J.M., Toro B., Chiong M., Hetz C and Lavandero S. (2013). Herp depletion protects from Biochim Biophys Acta. (BBA) - Molecular Cell Research. 1833:3295-3305. *#

Vidal R. and Hetz C. (2013). Unspliced XBP1 controls autophagy through FoxO1. Cell Res. 23(4):463-4. Editorial comment. *#

Matus S., Valenzuela V., Medina D. and Hetz C (2013). ER dysfunction and protein folding stress in ALS. Int. J Cell Biol. 2013:674751. *#

Cornejo VH, Pihán P., Vidal R., and Hetz C. (2013). Role of the UPR in organ physiology: Lessons from mouse models. IUBMB Life 65:962-75. *#

Cornejo VH. and Hetz C. (2013). The Unfolded Protein Response in Alzheimer’s disease. Semminars Immunol. 35(3):277-92. *#

Smaili SS, Quest AF, Hetz C, Lavandero S. (2013) Editorial: signaling in cell death, survival, proliferation and degeneration. Curr Mol Med. 2013 Feb 1;13(2):239-40*#


Hetz C. (2012).
The unfolded protein response: controlling cell fate decisions under ER stress and beyond. Nat Rev. Mol Cell Biol. 13(2):89-102. *#

Rodriguez D., Zamorano S., Lisbona F., Rojas D., Cubillos-Ruiz J., Armisen R., Henriquez D., Urra H., Cheng E., Letek M., Vaisar T., Irrazabal T., Gonzalez-Billault C., Letai A., Pimentel-Muiños F., Kroemer G. and Hetz C.. (2012). BH3-only proteins are part of a regulatory network that control the sustained signaling of the Unfolded Protein Response sensor IRE1.  EMBO J. 31(10):2322-35. *#

Vidal R., Figueroa A., Court F., Thielen P., Molina C., Wirth C., Caballero B., Kiffin R., Segura-Aguilar J., Cuervo AM, Glimcher L., and Hetz C. (2012). Targeting the UPR transcription factor XBP1 protects against Huntington’s disease through the regulation of FoxO1 and autophagy. Hum. Mol. Gen. 21(10):2245-62. *#

Rojas-Rivera D, Armisén R., Colombo A., Eguiguren A., Martínez G., Díaz A., Kiviluoto S., Rodríguez D., Patron M., Rizzuto R., Bultynck G., Concha M., Sierralta J., Stutzin A., and Hetz C. (2012). TMBIM3/GRINA is a novel Unfolded Protein Response (UPR) target gene that controls apoptosis through the modulation of ER calcium homeostasis. Cell Death Diff. 9:1013-26. *#

Zamorano S., Rojas-Rivera D., Lisbona F., Parra V., Court F., Villegas R., Cheng E., Korsmeyer S., Lavandero S., and Hetz C. (2012). A BAX/BAK and Cyclophilin D-independent Intrinsic Apoptosis Pathway. PLoS One :e37782. *#

Valenzuela V, Collyer E., Armentano D., Parsons G., Court F. and Hetz C. (2012). Activation of the Unfolded Protein Response enhances motor recovery after spinal cord injury. Cell Death Dis. 3, e272 *#

Torres M., Cartier L., Matamala J, Hernandez N., Woehvier U., and Hetz C. (2012). Altered Prion protein expression pattern in CSF as a biomarker for  Creutzfeldt-Jakob Disease. PLoS One .7:e36159. *#

Read D., Deepti A., Cawley K., Gupta A., Oommen D., Verfaillie T., Matus S., Agostinis P., Hetz C., Samali A. (2012). Perk-dependent Repression of miR-106b-25 Cluster is Required for ER stress-induced Apoptosis. Cell Death Dis. 28;3:e333 *#

Zuleta A., Rene L. Vidal R., Armentano D., Parsons G., and Hetz C. (2012). AAV-mediated delivery of the transcription factor XBP1s locally into the striatum reduces mutant Huntingtin aggregation in a mouse model of Huntington’s Disease. Biochem. Biophys Res Comm. 13:558-63.*#

Herrera R., Frischknecht R., Seidenbecher C., Gundelfinger E., Hetz C., Aylwin M., Schneider P., Quest A. And Leyton L. (2012). Astrocytic V3 Integrin Inhibits Neurite Outgrowth and Promotes Retraction of Neuronal Processes by Clustering Thy-1. PLoS One. 7: e34295. #

Martinez G. and Hetz C. (2012). Cell-nonautonomous control of the Unfolded Protein Response. EMBO Rep. *# 13:767-8. Editorial comment.

Urra H. and Hetz C. (2012). The ER in 4D: A novel stress pathway controlling endoplasmic reticulum membrane remodeling. Cell Death Diff. 19:1893-5. Editorial comment. *#

Nassif M and Hetz C (2012). Autophagy impairment: A crossroad between neurodegeneration and Tauopathies. BMC Biology. 10:78-81. Editorial comment.  *#

Klionsky and 1269 (Hetz C) others. (2012). Guidelines for the Use and Interpretation of Assays for Monitoring Autophagy in Higher Eukaryotes. Autophagy. 8:445.554. *#

Vidal R. and Hetz C. (2012). Crosstalk between the UPR and autophagy pathway contribute to handling cellular stress in neurodegenerative disease. Autophagy. 8:970-2. *#

Vidal R and Hetz C. (2012). Alleviating Secretory Pathway Stress in Huntington Disease. HD indights. 3:11-12.

Matus S, Castillo K, and Hetz C. (2012). Hormesis: Protecting neurons against cellular stress in Parkinson´s disease. Autophagy. 8:997-1001.*# Editorial comment. *#

Andreau C, Woehlbier U, Torres M, and Hetz C. (2012). Protein Disulfide Isomerases in Neurodegeneration: from disease mechanisms to biomedical applications. FEBS Lett.586:2826-34. *#


Castillo K, Rojas-Rivera D., Lisbona F., Caballero B., Nassif M., Court F., Schuck S., Ibar C., Walter P., Sierralta J., Glavic A., and Hetz C. (2011).
BAX inhibitor-1 regulates autophagy by controlling the IRE1/JNK branch of the unfolded protein response. EMBO J. 4465-78*#

Hetz C, Martino F, Rodriguez, and Glimcher LH. (2011).
The Unfolded Protein Response: Integrating stress signals through the stress sensor IRE1. Physiological Reviews. 91:1219-43. *#

Woehlbier U. and Hetz C. (2011).
Modulating stress responses by the UPRosome: A matter of life and death. Trends in Biochem Ssci (TiBS), 36:329-37. *#

Nassif M. and Hetz C. (2011).
Targeting autophagy in ALS: a complex mission. Autophagy. 7(4).*#

Matus S., Glimcher LH., and Hetz C. (2011).
Protein folding stress in neurodegenerative diseases: a glimpse into the ER. Curr Op. Cell Biol. 23:239-52.*#

Hetz C. and Glimcher LH. (2011)
Protein homeostasis networks in physiology and disease. Curr Op. Cell Biol. Editorial Overview. 23:123-5. *#

Torres M., Encina G., Soto C., and Hetz C. (2011).
Abnormal calcium homeostasis and protein folding stress at the ER: a common factor in familial and infectious Prion disorders. Commun Integr Biol. 4: 258 - 261. *#

Rodriguez D., Rojas D., and Hetz C. (2011).
The Endoplasmic Reticulum Gateway of Death. Biochimica et Biophysica Acta (BBA) - Molecular Cell Research. 1813:564-74. *#

Barrientos S.A., Martinez N.W., Yoo S., Jara, J.S., Zamorano S, Hetz, C., Twiss J.L. Alvarez J., Court F. A. (2011)
Axonal degeneration is mediated by the mitochondrial permeability transition pore. J. Neurosci. 31:966-978. *#

Klein A, Maldonado C., Vargas L., Robledo F., Perez de Arce K., Muñoz F., Hetz C., and Alvarez A., Zanlungo S.(2011).
Oxidative stress activates the c-Abl/p73 proapoptotic pathway in Niemann-Pick type C neurons Corresponding Neurobiol Dis. 41:209-18.

Vidal R., Caballero B., Couve A., and Hetz C. (2011).
Converging pathways in the occurrence of endoplasmic reticulum (ER) stress in Huntington’s disease. Curr. Mol. Med. 11:1-12 (Cover illustration) *#

Paula-Lima A, T. Adasme, A. Sebollela, C. Hetz, M. A. Carrasco, S. T. Ferreira and C. Hidalgo. (2011).
Amyloid β peptide oligomers stimulate RyR-mediated Ca2+release inducing mitochondrial fragmentation and prevent RyR-mediated dendritic spine remodeling produced by BDNF in hippocampal neurons. Antiox Red Sig. 14:1209-23.

Klein A, Mosqueira M, Martínez G, Robledo F, González M, Caballero B, Cancino GI, Alvarez AR, Hetz C, Zanlungo S.
Lack of activation of the unfolded protein response in mouse and cellular models of Niemann-Pick type C disease. Neurodegener Dis. 2011;8(3):124-8. Epub 2010 Aug 13.


Torres M., Castillo K., Arminsen R., Stutzin A., Soto C., and Hetz C. (2010).
Prion Protein Misfolding Affects Calcium Homeostasis and Sensitizes Cells to Endoplasmic Reticulum Stress. PlosOne. 5: e15658- e15658.*#

Sanjeev Gupta, Ayswaria Deepti, Shane Deegan, Fernanda Lisbona, Claudio Hetz and Afshin Samali. (2010).
HSP72 Protects Cells from ER Stress-induced Apoptosis via Enhancement of IRE1-XBP1 Signalling through a physical interaction. Plos Biology. 8: e1000410-e1000410.

Melissa Nassif, Soledad Matus, Karen Castillo, and Claudio Hetz (2010)
Amyotrophic lateral sclerosis pathogenesis: a journey through the secretory pathway. Antiox Red. Sig, 13:1955-89.


Matus S., Nassif M., Glimcher LH, and Hetz C. (2009).
XBP-1 deficiency in the nervous system revealed a homeostatic switch to activate autophagy. Autophagy. Nov 6;5(8). (Cover illustration) *#

Hetz C. and Glimcher L. (2009).
Fine tuning of the Unfolded Protein Response: Assembling the IRE1 interactome. Mol Cell. 35:551-61. *#

Hetz C, Thielen P, Matus S., M Nassif, Court F., Cuervo AM., Martinez G., Kiffin R., R.H. Brown and Glimcher L. (2009).
XBP-1 deficiency in the nervous system protects against amyotrophic lateral sclerosis by increasing autophagy. Genes & Dev. 23:2294–2306. (Cover illustration) *#
Commented in Genes Dev. (2009), 23:2294-306.

Lisbona F. and Hetz C. (2009).
Turning off the unfolded protein response: An interplay between the apoptosis machinery and ER stress signaling. Cell Cycle. 8:3-4.*#

Lisbona F., Rojas D., Thielen P., Zamorano S., Todd D., Martinon F., Glavic A., Kress C.,, Lin J., Walter P., Reed J., Glimcher L. and Hetz C. (2009).

BAX Inhibitor-1 is a negative regulator of the ER stress sensor IRE1. Mol. Cell. 33:679-691.*# (Cover illustration)
Commented in Mol Cell (2009), 33:669-70.

Hetz C. (2009).
The UPR as a survival factor of cancer cells: More than folding proteins? Leukemia Res. 33:880-882.*#


Matus S., Lisbona F., Torres M., Leon C., Thielen P., and Hetz C. (2008).
The stress rheostat: Interplay between the Unfolded Protein Response (UPR) and Autophagy in neurodegeneration. Curr. Mol. Med. 8:157-172. *#.

Hetz C. (2008).
Apoptosis, Autophagy and Necrosis: From mechanisms to biomedical applications. Curr. Mol. Med. 8(2):76-77 *#. Editorial comment.

Hetz C, Lee AH, Gonzalez-Romero D., Thielen P., Castilla J., Soto C. and Glimcher L. (2008).
The Unfolded Protein Response transcription factor XBP-1 does not influence Prion replication or pathogenesis. Proc. Natl. Acad Sci. 105: 757-62 *#

Hetz C. and Glimcher L. (2008)
The deadly job of night Killer: New regulatory roles of the BCL-2 family in organelle physiology. Trends in Cell Biol. 2008 Jan;18(1):38-44. (Cover illustration)

Hetz C. and Glimcher L. (2008).
The UPRosome and XBP-1: mastering secretory cell function. Curr. Immunol Rev. 4:1-10*#.


Steele A., Hetz C., Yi C., Jackson W., Borkowski A., Yuan J., Wollmann R., and Lindquist S. (2007).
Prion pathogenesis is independent of Caspase-12. Prion. 4:1-5. *#

Steele A., King O., Jackson W., Hetz C., Borkowski A., Thielen P., Wollmann R., and Lindquist S. (2007).
Diminishing apoptosis by deletion of Bax or overexpression of Bcl-2 does not protect against infectious prion toxicity in vivo (2007) J. of Neuroscience. 27: 13022-7. *

Hetz C. (2007).
ER stress signaling: Form adaptation to irreversible cellular damage. Antiox Redox Signal. 9:1-12. *#

Hetz C. Thielen P., Fisher J., Pacinelli, P., Brown, R., Korsmeyer S. and Glimcher L. (2007).
The Pro-apoptotic BCL-2 Family Member BIM Mediates Motoneuron Loss in a Model of Amyotrophic Lateral Sclerosis. Cell Death and Diff. 14(7):1386-1389.*#

Hetz, C., Castilla, J. and Soto C. (2007).
Perturbation of endoplasmic reticulum homeostasis facilitates prion replication. J. Biol. Chem. 282:12725-33. *#


Hetz, C, Bernasconi, P., Fisher, J., Lee, A., Bassik, M., Antonsson, B., Brandt, G., Iwakoshi, N,. Schinzel, A., Glimcher, L. and Korsmeyer, S. (2006).
Proapoptotic BAX and BAK modulate the unfolded protein response by a direct interaction with IRE1alpha. Science.312: 572-6. #

Commented in Nat. Rev. Cell Biol. (2006)7:388 Handling the stress; ACS Chem. Biol. (2006)1:188. Stressed out cells.

Hetz, C. and Soto, C. 2006.
Emerging roles of the Unfolded protein response signaling in physiology and disease. Curr. Mol. Med. 6: 1-3. Editorial comment as executive editor.*#

Hetz, C. and Soto, C. 2006.
Stressing out the ER: A Role of unfolded protein response in prion-related disorders Curr. Mol. Med. 6:37-43.*#


Hetz, C., Vitte, P., Bombrun, A., Rostovtseva, T., Montessuit, S., Hiver, A., Schwarz, M., Church, D., Korsmeyer, S., Martinou, J.C. and Antonsson, B. 2005.
Bax channel inhibitors prevent cytochrome c release from mitochondria and protect neurons in a model of global brain ischemia. J. Biol. Chem. 280: 42960-42970.*

Hetz, C., Torres, V., and Quest A. 2005.
Beyond apoptosis: Non-apoptotic cell death in physiology and disease. Biochem. Cell. Biol. 83:579-588.*

Schinzel, A., Takeuchi, O., Huang, Z., Fisher, J., Zhou, Z., Rubens J., Hetz, C. Danial, N., Moskowits, M., and Korsmeyer, S. 2005.
Cyclophilin D is an essential component of mitochondrial permeability transition and mediates neuronal cell death after focal cerebral ischemia. Proc. Natl Acad. Sci. U.S.A. 102:12005-12010.

Castilla, J., Saa, P., Hetz, C. and Soto, C. 2005.
In vitro generation of infectious scrapie prions. Cell. 121:195-206.*

Commented in Cell (2005)121:155-162. From microbes to prions: The final proof of the prion hypothesis; Science (2005)421:3. Proof positive for prions; Nature (2005)432:39. Prion progress; Nature Med. (2005)11:480. Nothing but prion.

Hetz C., Russelakis, M., Wälchli S., Carboni S., Vial-Knecht E., Maundrell K. and Soto, C. 2005.
The disulfide isomerase Grp58 is a protective factor against prion neurotoxicity. J. Neurosci. 25:2793-2802.*


Russelakis, M., Hetz C., Maundrell K., and Soto, C. 2004.
Prion replication alters the distribution of synaptophysin and caveolin 1 in neuronal lipid rafts. Am. J. Pathol. 165:1839-1848.*

Castilla C., Hetz C., Soto C. 2004.
Molecular mechanisms of neurotoxicity of pathological Prion protein. Curr. Mol. Med. 4:397-403. *


Hetz C., Russelakis, M., Maundrell, K. Castilla, J. and Soto, C. 2003.
Caspase-12 and endoplasmic reticulum stress mediate neurotoxicity of pathological prion protein. EMBO J. 22:5435-5445. *

Commented in Science, STKE (2003)302:1007. Prions stress out the ER.

Hetz, C., Maundrell, K. and Soto, C. 2003.
Is loss of function of the prion protein the cause of Prion Disorders? Trends Mol. Med. 9:237-243.*

Hetz, C. and Soto, C. 2003.
Prion Misfolding and Disease: The Case of Prion Protein. Cell. Mol. Life Sci. 60:133-1432.*

Hetz C., Benavent, S. Bieler, S. and Soto, C. 2003.
Prion Protein Misfolding and Brain Degeneration. Curr. Med. Chem. 3:137-147.*


Hetz, C., Hunn, M., Rojas, P. Leyton, L., and Quest, A. F. 2002.
Caspase-dependent Initiation of Apoptosis and Necrosis by the Fas Recetor: A Role for the Lipid Second Messenger Ceramide in Initiation of FasL-induced Necrosis. J. Cell Sci. 115: 4671-83.*

Commented in J. Cell. Sci. (2002)115:e2304. Apoptosis vs necrosis: ceramide decides it. and in Science, STKE (2002), Issue 158: 414. A Back-up death plan.

Hetz, C. Bono, M., Barros, F. and Lagos, R.. 2002.
Microcin E492, a Channel-forming Bacteriocin from Klebsiella pneumoniae, Induces Apoptosis in Some Human Cell Lines. Proc. Natl. Acad. Sci USA. 99:2696-2701. *


Leyton, L., Schneider, P., Labra, C., Ruegg, C., Hetz, C., Quest, A. and Brown C. 2001.
Thy-1 binds to the integrin 3 on astrocytes and triggers formation of focal contact sites. Curr. Biol. 11:1028-38.*

Barros, F., Stutzin, A., Calixto, A., Catalán, M., Castro, J., Hetz, C., and Hermosilla, T. 2001.
Non-selective Cation Channels as Effectors of Free Radical-induced Liver Cell Necrosis. Hepatology. 33:114-22.*

Lagos, R., Baeza, M., Corsini, G., Hetz, C., Strahsburger, E., Castillo J., Vergara C., and Monasterio O. 2001.
Structure, Organization and Characterization of the Gene Cluster Involved in the Production of Microcin E492, a Channel-Forming Bacteriocin. Mol. Microbiol. 42: 229-244.*

Book chapters

Bargsted L., Vidal R., Hetz C., and Matus M. (2015) Dual role of autophagy in neurodegenerative diseases: The case of amyotrophic lateral sclerosis. Springer editorial. Book entitled “Toxicity and Autophagy in Neurodegenerative Disorders”. In press. *#

Nassif M, Danilo Medinas, Karen Castillo, Camila Gherardelli, and Claudio Hetz. (2013).
When the good turns bad: Challenges in the targeting of autophagy in neurodegenerative disease. Book Chapter 4-volume series, "Autophagy: Cancer, Other Pathologies, Infection, Inflammation and Immunity" Elsevier Publishing Company. In press.

Galleguillos D., Matus S. Valenzuela V., Valdes P., Martinez A., Nassif M., Torres M., Martinez G., and Hetz C. 2009. Targeting endoplasmic reticulum stress pathways to alleviate neurological disorders associated with protein misfolding. E-book series. Bentham Science Publishers.

Rojas D., Caballero, B., Zamorano S., Lisbona F., and Hetz C. 2010. Alternative Functions of the BCL-2
Protein Family at the Endoplasmic Reticulum. Bioscience publishers. Adv Exp Med Biol. 2010;687:33-47.

Hetz C. BCL-2 protein family. 2010.
Essential regulators of cell death. Preface. Bioscience publishers. Adv Exp Med Biol. 2010;687:vii-viii.

Matus S., Thielen P., Nassif M., Torres M., and Hetz C (2008)
Targeting stress pathways to treat neurological disorders linked to protein misfolding. Protein Misfolding. Nova Science Publishers, Inc. Editor Cian B. O'Doherty and Adam C. Byrne *#

Russelakis-Carneiro, M., Hetz, C., Castilla, J. and Soto, C. (2004)
Neurodegeneration induced by prion protein misfolding. In “Neuroprotection: Models, Mechanisms, Therapies” M. Bahr (Ed.). Wiley-VCH.*

Morales R., Hetz C. and Soto C. (2004).
Signaling Pathways Controlling Prion Neurotoxicity: Role of Endoplasmic reticulum stress-mediated Apoptosis. Neurodegeneration in Prion Disorders. Klewer. Editor: David Brown. *


Book editor: e-book “Protein Misfolding Disorders: A Trip into the ER”. 2009. Bentham Science Publishers.
In press

Book editor: “BCL2 Protein Family: Functions beyond Apoptosis”. 2010. Bioscience publishers.
In press