Abstract:
:In the lysosomal storage disorder Gaucher disease (GD), glucosylceramide (GlcCer) accumulates due to the defective activity of glucocerebrosidase. A subset of GD patients develops neuropathology. We now show mislocalization of Limp2-positive puncta and a large reduction in the number of Lamp1-positive puncta, which are associated with impaired tubulin. These changes occur at an early stage in animal models of GD, prior to development of overt symptoms and considerably earlier than neuronal loss. Altered lysosomal localization and cytoskeleton disruption precede the neuroinflammatory pathways, axonal dystrophy and neuronal loss previously characterized in neuronal forms of GD.
journal_name
FEBS Lettjournal_title
FEBS lettersauthors
Zigdon H,Meshcheriakova A,Farfel-Becker T,Volpert G,Sabanay H,Futerman AHdoi
10.1002/1873-3468.12591subject
Has Abstractpub_date
2017-03-01 00:00:00pages
774-783issue
5eissn
0014-5793issn
1873-3468journal_volume
591pub_type
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