Human INCL fibroblasts display abnormal mitochondrial and lysosomal networks and heightened susceptibility to ROS-induced cell death

Infantile Neuronal Ceroid Lipofuscinosis (INCL) is a pediatric neurodegenerative disorder characterized by progressive retinal and central nervous system deterioration during infancy. This lysosomal storage disorder results from a deficiency in the Palmitoyl Protein Thioesterase 1 (PPT1) enzyme—a ly...

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Detalles Bibliográficos
Autores principales: Balouch, Bailey, Nagorsky, Halle, Pham, Truc, LaGraff, James Thai, Chu-LaGraff, Quynh
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2021
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7872282/
https://www.ncbi.nlm.nih.gov/pubmed/33561134
http://dx.doi.org/10.1371/journal.pone.0239689

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7872282/
https://www.ncbi.nlm.nih.gov/pubmed/33561134
http://dx.doi.org/10.1371/journal.pone.0239689

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