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A saposin deficiency model in Drosophila: Lysosomal storage, progressive neurodegeneration and sensory physiological decline

Saposin deficiency is a childhood neurodegenerative lysosomal storage disorder (LSD) that can cause premature death within three months of life. Saposins are activator proteins that promote the function of lysosomal hydrolases that mediate the degradation of sphingolipids. There are four saposin pro...

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Detalles Bibliográficos
Autores principales: Hindle, Samantha J., Hebbar, Sarita, Schwudke, Dominik, Elliott, Christopher J.H., Sweeney, Sean T.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Academic Press 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5319729/
https://www.ncbi.nlm.nih.gov/pubmed/27913291
http://dx.doi.org/10.1016/j.nbd.2016.11.012