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Autophagy Inhibitors Do Not Restore Peroxisomal Functions in Cells With the Most Common Peroxisome Biogenesis Defect

Peroxisome biogenesis disorders within the Zellweger spectrum (PBD-ZSDs) are most frequently associated with the c.2528G>A (p.G843D) mutation in the PEX1 gene (PEX1-G843D), which results in impaired import of peroxisomal matrix proteins and, consequently, defective peroxisomal functions. A recent...

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Detalles Bibliográficos
Autores principales: Klouwer, Femke C. C., Falkenberg, Kim D., Ofman, Rob, Koster, Janet, van Gent, Démi, Ferdinandusse, Sacha, Wanders, Ronald J. A., Waterham, Hans R.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8047214/
https://www.ncbi.nlm.nih.gov/pubmed/33869228
http://dx.doi.org/10.3389/fcell.2021.661298