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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Bibliographic Details
Main Authors: Samantha J. Hindle, Sarita Hebbar, Dominik Schwudke, Christopher J.H. Elliott, Sean T. Sweeney
Format: Article
Language:English
Published: Elsevier 2017-02-01
Series:Neurobiology of Disease
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996116302807