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个人简介
Our research is focused on understanding redox processes determining protein folding and conformational transitions that contribute to neuronal morphogenesis and cellular signaling under physiological and pathological conditions. Using a combination of genetic and proteomic tools, we currently work to define proteostasis pathways and post-translational modifications to proteins mediating intercellular communication and synaptic plasticity. We are particularly interested in investigating the contribution of protein disulfide isomerases (PDIs) in the endoplasmic reticulum, a major compartment involved in protein synthesis and secretion, to neurodevelopment and their dysfunction in neurological disorders. We have described PDIs function in folding of the Prion protein and characterized PDIs mutants in amyotrophic lateral sclerosis (ALS) and intellectual disability (ID). We are now developing genetically engineered mouse lines as novel genetic models of these diseases. Moreover, we are mapping PDIs function in the nervous system to understand selective neuronal vulnerability in different pathological states. Finally, my group is committed to move forward translational strategies using small molecules and gene therapy to restore proteostasis as therapeutics for nervous system pathologies.
研究兴趣
论文共 54 篇作者统计合作学者相似作者
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Janina Borgonovo,Camilo Allende-Castro,Danilo B. Medinas, Deyanira Cárdenas, María Paz Cuevas,Claudio Hetz,Miguel L. Concha
Cell and Tissue Researchno. 1 (2024): 21-38
Danilo Medinas,Pablo Rozas, Harrison Baker,Julio Cesar Ferreira, Fadzai Teramayi, Patricia Ojeda-Provoste, Giselle Espinosa, Gabriel Quiroz, Ricardo Pestana,Fernando Melleu,Darwin Contreras, Felipe Godoy,
FREE RADICAL BIOLOGY AND MEDICINE (2023): S162-S162
The EMBO journalno. 22 (2022): e111952-e111952
The EMBO Journalno. 22 (2022)
Claudia Duran-Aniotz, Catalina Rivera-Krstulovic,Natalia Poblete,Álvaro O. Ardiles, Mei Li Díaz,Carleen Mae P. Sabusap, Yannis Gerakis,Felipe Cabral Miranda,Javier Diaz,Matias Fuentealba, Ernesto Muñoz,Sandra Espinoza,
Molecular therapy : the journal of the American Society of Gene Therapyno. 7 (2022): 2240-2256
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