Cargando…
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: | Breiden, Bernadette, Sandhoff, Konrad |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2020
|
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 |
Ejemplares similares
-
Acid Sphingomyelinase, a Lysosomal and Secretory Phospholipase C, Is Key for Cellular Phospholipid Catabolism
por: Breiden, Bernadette, et al.
Publicado: (2021) -
Secondary ganglioside GM2 accumulation in mucopolysaccharidoses
por: Sandhoff, Konrad
Publicado: (2021) -
Inhibition of Lysosome Membrane Recycling Causes Accumulation of Gangliosides that Contribute to Neurodegeneration
por: Boutry, Maxime, et al.
Publicado: (2003) -
Acid sphingomyelinase activity is regulated by membrane lipids and facilitates cholesterol transfer by NPC2
por: Oninla, Vincent O., et al.
Publicado: (2014) -
Role of endosomal membrane lipids and NPC2 in cholesterol transfer and membrane fusion
por: Abdul-Hammed, Misbaudeen, et al.
Publicado: (2010)