Cargando…
Correction of oxidative stress enhances enzyme replacement therapy in Pompe disease
Pompe disease is a metabolic myopathy due to acid alpha‐glucosidase deficiency. In addition to glycogen storage, secondary dysregulation of cellular functions, such as autophagy and oxidative stress, contributes to the disease pathophysiology. We have tested whether oxidative stress impacts on enzym...
Autores principales: | Tarallo, Antonietta, Damiano, Carla, Strollo, Sandra, Minopoli, Nadia, Indrieri, Alessia, Polishchuk, Elena, Zappa, Francesca, Nusco, Edoardo, Fecarotta, Simona, Porto, Caterina, Coletta, Marcella, Iacono, Roberta, Moracci, Marco, Polishchuk, Roman, Medina, Diego Luis, Imbimbo, Paola, Monti, Daria Maria, De Matteis, Maria Antonietta, Parenti, Giancarlo |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
John Wiley and Sons Inc.
2021
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8573602/ https://www.ncbi.nlm.nih.gov/pubmed/34606154 http://dx.doi.org/10.15252/emmm.202114434 |
Ejemplares similares
-
Abnormal mannose-6-phosphate receptor trafficking impairs recombinant alpha-glucosidase uptake in Pompe disease fibroblasts
por: Cardone, Monica, et al.
Publicado: (2008) -
Pathogenesis of Mucopolysaccharidoses, an Update
por: Fecarotta, Simona, et al.
Publicado: (2020) -
AAV-mediated transcription factor EB (TFEB) gene delivery ameliorates muscle pathology and function in the murine model of Pompe Disease
por: Gatto, Francesca, et al.
Publicado: (2017) -
Carnitine is a pharmacological allosteric chaperone of the human lysosomal α-glucosidase
por: Iacono, Roberta, et al.
Publicado: (2021) -
The Pharmacological Chaperone N-butyldeoxynojirimycin Enhances Enzyme Replacement Therapy in Pompe Disease Fibroblasts
por: Porto, Caterina, et al.
Publicado: (2009)