Endolysosomal N-glycan processing is critical to attain the most active form of the enzyme acid alpha-glucosidase

Acid alpha-glucosidase (GAA) is a lysosomal glycogen-catabolizing enzyme, the deficiency of which leads to Pompe disease. Pompe disease can be treated with systemic recombinant human GAA (rhGAA) enzyme replacement therapy (ERT), but the current standard of care exhibits poor uptake in skeletal muscl...

Descripción completa

Detalles Bibliográficos
Autores principales: Selvan, Nithya, Mehta, Nickita, Venkateswaran, Suresh, Brignol, Nastry, Graziano, Matthew, Sheikh, M. Osman, McAnany, Yuliya, Hung, Finn, Madrid, Matthew, Krampetz, Renee, Siano, Nicholas, Mehta, Anuj, Brudvig, Jon, Gotschall, Russell, Weimer, Jill M., Do, Hung V.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: American Society for Biochemistry and Molecular Biology 2021
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8191302/
https://www.ncbi.nlm.nih.gov/pubmed/33971197
http://dx.doi.org/10.1016/j.jbc.2021.100769

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8191302/
https://www.ncbi.nlm.nih.gov/pubmed/33971197
http://dx.doi.org/10.1016/j.jbc.2021.100769

Ejemplares similares