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| 1 | Gene therapy in Parkinson's disease: targeting the endplasmic reticulum proteostasis network显示文摘Parkinson’s disease(PD)is the second most common neurodegenerative disease affecting 1%of the population over 60 years of age.The 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.There is no cure for PD and available treatments only aim to restore dopamine deficits.Administration of the dopamine precursor Levodopa(L-DOPA)is the main temporal palliative treatment that increases overall dopamine levels.However,its chronic use limits its effectiveness and generates a number of adverse effects such as debilitating dyskinesia. | Valentina Castillo Gabriela Mercado Claudio Hetz | 2015 | Neural Regeneration Research2015,10,7: | 2 |
| 2 | ERp57 in neurodegeneration and regeneration显示文摘The protein disulfide isomerases(PDIs)family has a central function in the folding of proteins synthetized through the secretory pathway.ERp57,also known as Grp58 or PDIA3,is one of the main studied members of this family.ERp57 catalyzes the formation,disruption and isomerization of disulfide bonds of glycoproteins mediated by a cooperative interaction with | Leslie Bargsted Claudio Hetz Soledad Matus | 2016 | Neural Regeneration Research2016,11,2: | 1 |
| 3 | An ERcentric view of Parkinson’s disease显示文摘 | Gabriela Mercado Pamela Valdés Claudio Hetz | 2013 | Trends in Molecular Medicine2013,,: | 1 |
| 4 | Bax channel inhibitors prevent mitochondrion - mediated apoptosis and protect neurons in a model of global brain ischemia显示文摘 | Claudio Hetz Pierre - Alain Vitte Agnes Bombrun | 2005 | J Biol Chem2005,280,42: | 1 |
| 5 | Proapoptotic RECS1: a requisite gateway to lysosomal dysfunction and death显示文摘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 cargo.The 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 cell.For 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. | Philippe Pihán Mateus Milani Claudio Hetz | 2022 | Neural Regeneration Research2022,17,12: | 0 |
| 6 | Bursting the unfolded protein response accelerates axonal regeneration显示文摘Peripheral neuropathies refer to a group of conditions in which the peripheral nervous system(PNS)is damaged.These pathological state are are associated with weakness,pain,and loss of motor and sensory control.More than 100 types of peripheral neuropathies have been identified,with distinct symptoms and prognosis classified according to the type of damage to the nerves.Injury to peripheral nerves results in disabling loss of sensory and motor func- | Maritza Onate | 2016 | Neural Regeneration Research2016,11,6: | 0 |
| 7 | Therapeutic potential of insulin-like growth factor 2 in Huntington's disease: controlling proteostasis to alleviate the load of misfolded protein显示文摘Huntington's disease(HD) is an inherited autosomal dominant neurodegenerative disorder characterized by the development of adult-onset motor dysfunction,psychiatric disturbances and intellectual decline.HD 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. | Paulina Troncoso-Escudero Claudio Hetz Rene L.Vidal | 2021 | Neural Regeneration Research2021,16,8: | 0 |
| 8 | Preventing brain aging by the artificial enforcement of the unfolded protein response:future directions显示文摘As the life expectancy of the world’s population increases,age-related diseases are emerging as one of the greatest problems facing modern society.The 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. | Felipe Cabral-Miranda Claudio Hetz | 2024 | Neural Regeneration Research2024,19,2: | 0 |