Replacement of α-galactosidase A in Fabry disease: effect on fibroblast cultures compared with biopsied tissues of treated patients

The function and intracellular delivery of enzyme therapeutics for Fabry disease were studied in cultured fibroblasts and in the biopsied tissues of two male patients to show diversity of affected cells in response to treatment. In the mutant fibroblasts cultures, the final cellular level of endocyt...

Descripción completa

Detalles Bibliográficos
Autores principales: Keslová-Veselíková, Jana, Hůlková, Helena, Dobrovolný, Robert, Asfaw, Befekadu, Poupětová, Helena, Berná, Linda, Sikora, Jakub, Goláň, Lubor, Ledvinová, Jana, Elleder, Milan
Formato: Texto
Lenguaje:English
Publicado: Springer-Verlag 2008
Materias:
Original Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2956889/
https://www.ncbi.nlm.nih.gov/pubmed/18351385
http://dx.doi.org/10.1007/s00428-008-0586-9

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2956889/
https://www.ncbi.nlm.nih.gov/pubmed/18351385
http://dx.doi.org/10.1007/s00428-008-0586-9

Ejemplares similares