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Cathepsin B-associated Activation of Amyloidogenic Pathway in Murine Mucopolysaccharidosis Type I Brain Cortex

Mucopolysaccharidosis type I (MPS I) is caused by genetic deficiency of α-l-iduronidase and impairment of lysosomal catabolism of heparan sulfate and dermatan sulfate. In the brain, these substrates accumulate in the lysosomes of neurons and glial cells, leading to neuroinflammation and neurodegener...

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Detalles Bibliográficos
Autores principales: Viana, Gustavo Monteiro, Gonzalez, Esteban Alberto, Alvarez, Marcela Maciel Palacio, Cavalheiro, Renan Pelluzzi, do Nascimento, Cinthia Castro, Baldo, Guilherme, D’Almeida, Vânia, de Lima, Marcelo Andrade, Pshezhetsky, Alexey V., Nader, Helena Bonciani
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7073069/
https://www.ncbi.nlm.nih.gov/pubmed/32093427
http://dx.doi.org/10.3390/ijms21041459