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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...
Autores principales: | Frustaci, Andrea, Verardo, Romina, Scialla, Rossella, Bagnato, Giulia, Verardo, Margherita, Alfarano, Maria, Russo, Matteo A. |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2022
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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 |
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