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Downregulation of Mannose-6-Phosphate Receptors in Fabry Disease Cardiomyopathy: A Potential Target for Enzyme Therapy Enhancement

Background: The efficacy of enzyme replacement therapy (ERT) in mobilizing globotryaosylceramide (GB-3) from Fabry cardiomyocytes is limited. The mechanism involved is still obscure. Methods: Assessment of M6Pr, M6Pr-mRNA, and Ubiquitin has been obtained by Western blot analysis and real-time PCR of...

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Detalles Bibliográficos
Autores principales: Frustaci, Andrea, Verardo, Romina, Scialla, Rossella, Bagnato, Giulia, Verardo, Margherita, Alfarano, Maria, Russo, Matteo A.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9502235/
https://www.ncbi.nlm.nih.gov/pubmed/36143092
http://dx.doi.org/10.3390/jcm11185440