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Preventing brain aging by the artificial enforcement of the unfolded protein response:future directions

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Neural Regeneration Research 2024年第2期19卷 393-394页

作者： Felipe Cabral-Miranda claudio hetz Instituto de Ciências Biomédicas Universidade Federal do Rio de JaneiroRio de JaneiroBrazil Center for Geroscience Brain Health and MetabolismSantiagoChile、Biomedical Neuroscience InstituteFaculty of MedicineUniversity of ChileSantiagoChile、Program of Cellular and Molecular BiologyInstitute of Biomedical SciencesFaculty of MedicineUniversity of ChileSantiagoChile、Buck Institute for Research on AgingNovatoCAUSA

As the life expectancy of the world’s population increases,age-related diseases are emerging as one of the greatest problems facing modern *** onset of dementia and neurodegenerative diseases is strictly dependent on aging as a major risk factor and has a profound impact on various aspects of the lives of individuals and their families.

关键词： diseases aging directions

Proapoptotic RECS1: a requisite gateway to lysosomal dysfunction and death

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Neural Regeneration Research 2022年第12期17卷 2695-2696页

作者： Philippe Pihán Mateus Milani claudio hetz Biomedical Neuroscience Institute Faculty of MedicineUniversity of ChileSantiagoChile Center for Geroscience Brain Health and Metabolism(GERO)SantiagoChile Program of Cellular and Molecular Biology Institute of Biomedical SciencesUniversity of ChileSantiagoChile Buck Institute for Research on Aging NovatoCAUSA

For a long time since their discovery by Christian de Duve in the 1950s,lysosomes have been referred to almost exclusively as passive garbage bags;the endpoint in the degradation of intra-and extracellular *** catabolic function of lysosomes is accomplished by an array of more than 60 acid hydrolases,which together break down a wide variety of biological macromolecules,including proteins,lipids,carbohydrates,and nucleic acids,for reutilization in the metabolic processes of the *** their optimal function,these enzymes require an acidic intraluminal pH of~4.5,which is maintained by the joint action of a proton pump,the vacuolar H+-ATPase,and several ion channels embedded in the lysosomal limiting membrane.

关键词： maintained limiting function

ERp57 in neurodegeneration and regeneration

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Neural Regeneration Research 2016年第2期11卷 232-233页

作者： Leslie Bargsted claudio hetz Soledad Matus Neurounion Biomedical Foundation CENPAR Santiago Chile Biomedical Neuroscience Institute Faculty of Medicine University of Chile Santiago Chile Center for Geroscience Brain Health and Metabolism Institute of Biomedical Sciences Center for Molecular Studies of the Cell Program of Cellular and Molecular Biology University of ChileSantiago Chile Department of Immunology and Infectious Diseases Harvard School of Public Health

The protein disulfide isomerases（PDIs）family has a central function in the folding of proteins synthetized through the secretory ***57,also known as Grp58 or PDIA3,is one of the main studied members of this family.

关键词： ERp57 in neurodegeneration and regeneration PDI

Gene therapy in Parkinson's disease: targeting the endplasmic reticulum proteostasis network

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Neural Regeneration Research 2015年第7期10卷 1053-1054页

作者： Valentina Castillo Gabriela Mercado claudio hetz Biomedical Neuroscience Institute Faculty of MedicineUniversity of Chile Program of Cellular and Molecular Biology Center for Molecular Studies of the Cell Institute of Biomedical SciencesUniversity of Chile Department of Immunology and Infectious Diseases Harvard School of Public Health

Parkinson’s disease（PD）is the second most common neurodegenerative disease affecting 1%of the population over 60 years of *** progressive degeneration of dopaminergic neurons at the substantia nigra pars compacta（SNpc）results in a severe and gradual depletion of dopamine content in the striatum,a phenomena that is responsible for the characteristic motor symptoms of this disease.

关键词： gene Gene therapy in Parkinson’s disease targeting the endplasmic reticulum proteostasis network

Therapeutic potential of insulin-like growth factor 2 in Huntington's disease: controlling proteostasis to alleviate the load of misfolded protein

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Neural Regeneration Research 2021年第8期16卷 1564-1565页

作者： Paulina Troncoso-Escudero claudio hetz Rene L.Vidal Biomedical Neuroscience Institute Faculty of MedicineUniversity of ChileSantiagoChile Center for Geroscience Brain Health and MetabolismSantiagoChile Program of Cellular and Molecular Biology Institute of Biomedical SciencesUniversity of ChileSantiagoChile Center for Integrative Biology Faculty of SciencesUniversity MayorChile Buck Institute for Research on Aging NovatoCAUSA

Huntington's disease(HD) is an inherited autosomal dominant neurodegenerative disorder characterized by the development of adult-onset motor dysfunction,psychiatric disturbances and intellectual *** is associated with an expansion of CAG repeat sequence in the huntingtin gene(Htt).Exon 1 of Htt normally contains between 6 to 35 CAG repeats,whereas in patients affected with HD it contains more than 40 trinucleotides.

关键词： Huntington Therapeutic stasis