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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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Detalhes bibliográficos
Publicado no:Neurobiol Dis
Main Authors: Hindle, Samantha J., Hebbar, Sarita, Schwudke, Dominik, Elliott, Christopher J.H., Sweeney, Sean T.
Formato: Artigo
Idioma:Inglês
Publicado em: Academic Press 2017
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC5319729/
https://ncbi.nlm.nih.gov/pubmed/27913291
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.nbd.2016.11.012
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