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Mechanism of Secondary Ganglioside and Lipid Accumulation in Lysosomal Disease
Gangliosidoses are caused by monogenic defects of a specific hydrolase or an ancillary sphingolipid activator protein essential for a specific step in the catabolism of gangliosides. Such defects in lysosomal function cause a primary accumulation of multiple undegradable gangliosides and glycosphing...
Autores principales: | , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7178057/ https://www.ncbi.nlm.nih.gov/pubmed/32272755 http://dx.doi.org/10.3390/ijms21072566 |