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Defects in sarcolemma repair and skeletal muscle function after injury in a mouse model of Niemann-Pick type A/B disease

BACKGROUND: Niemann-Pick disease type A (NPDA), a disease caused by mutations in acid sphingomyelinase (ASM), involves severe neurodegeneration and early death. Intracellular lipid accumulation and plasma membrane alterations are implicated in the pathology. ASM is also linked to the mechanism of pl...

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Detalles Bibliográficos
Autores principales: Michailowsky, V., Li, H., Mittra, B., Iyer, S. R., Mazála, D. A. G., Corrotte, M., Wang, Y., Chin, E. R., Lovering, R. M., Andrews, N. W.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6320626/
https://www.ncbi.nlm.nih.gov/pubmed/30611303
http://dx.doi.org/10.1186/s13395-018-0187-5