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Neural Regeneration Research 2023年第10期18卷 2156-2160页

作者： María A.pajares Elena Hernández-Gerez Milos pekny dolores pérez-sala Department of Structural and Chemical Biology Centro de Investigaciones Biológicas Margarita SalasMadridSpain Laboratory of Astrocyte Biology and CNS Regeneration Center for Brain RepairDepartment of Clinical NeuroscienceInstitute of Neuroscience and PhysiologySahlgrenska Academy at the University of GothenburgGothenburgSweden University of Newcastle NewcastleNSWand the Florey Institute of Neuroscience and Mental HealthParkvilleVICAustralia

Alexander disease is a rare neurodegenerative disorder caused by mutations in the glial fibrillary acidic protein,a type III intermediate filament protein expressed in *** early(infantile or juvenile)and adult onsets of the disease are known and,in both cases,astrocytes present characteristic aggregates,named Rosenthal *** are spread along the glial fibrillary acidic protein sequence disrupting the typical filament network in a dominant *** the presence of aggregates suggests a proteostasis problem of the mutant forms,this behavior is also observed when the expression of wild-type glial fibrillary acidic protein is ***,several isoforms of glial fibrillary acidic protein have been described to date,while the impact of the mutations on their expression and proportion has not been exhaustively ***,the posttranslational modification patterns and/or the protein-protein interaction networks of the glial fibrillary acidic protein mutants may be altered,leading to functional changes that may modify the morphology,positioning,and/or the function of several organelles,in turn,impairing astrocyte normal function and subsequently affecting *** particular,mitochondrial function,redox balance and susceptibility to oxidative stress may contribute to the derangement of glial fibrillary acidic protein mutant-expressing *** study the disease and to develop putative therapeutic strategies,several experimental models have been developed,a collection that is in constant *** fact that most cases of Alexander disease can be related to glial fibrillary acidic protein mutations,together with the availability of new and more relevant experimental models,holds promise for the design and assay of novel therapeutic strategies.

关键词： astrocytes endoplasmic reticulum stress glial fibrillary acidic protein mutants metabolism misassembly misfolding neurodegeneration oxidative stress posttranslational modifications unfolded protein response

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