The New Pharmacological Chaperones PBXs Increase α-Galactosidase A Activity in Fabry Disease Cellular Models

Fabry disease is an X-linked multisystemic disorder caused by the impairment of lysosomal α-Galactosidase A, which leads to the progressive accumulation of glycosphingolipids and to defective lysosomal metabolism. Currently, Fabry disease is treated by enzyme replacement therapy or the orally admini...

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Detalles Bibliográficos
Autores principales: Besada, Pedro, Gallardo-Gómez, María, Pérez-Márquez, Tania, Patiño-Álvarez, Lucía, Pantano, Sergio, Silva-López, Carlos, Terán, Carmen, Arévalo-Gómez, Ana, Ruz-Zafra, Aurora, Fernández-Martín, Julián, Ortolano, Saida
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2021
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8699827/
https://www.ncbi.nlm.nih.gov/pubmed/34944500
http://dx.doi.org/10.3390/biom11121856

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8699827/
https://www.ncbi.nlm.nih.gov/pubmed/34944500
http://dx.doi.org/10.3390/biom11121856

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