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Mark Cooper, Andrey Y. Abramov, Matthew Gegg, Anthony H. Gaucher disease is caused by mutations in the glucocerebrosidase gene, which encodes the lysosomal hydrolase glucosylceramidase.
Therefore there is an urgent need to understand the mechanisms by which glucocerebrosidase mutations predispose to neurodegeneration to facilitate development of novel treatments. Glucosylceramidase protein and enzyme activity levels were assayed.
Oxidative stress was assayed by single cell imaging of dihydroethidium. We hypothesized that treatment with the molecular chaperone ambroxol hydrochloride would improve these biochemical abnormalities. In conclusion, glucocerebrosidase mutations are associated with reductions in glucosylceramidase activity and evidence of oxidative stress. Ambroxol treatment significantly increases glucosylceramidase activity and reduces markers of oxidative stress in cells bearing glucocerebrosidase mutations.
The autophagy—lysosome system plays a key role in degrading the misfolded proteins that form the abnormal protein accumulations that occur in the common late onset neurodegenerative diseases.
Markers of dysfunctional autophagy have been described in motor neuron disease spinal cord Otomo et al. In addition cell biology studies have demonstrated that inhibition of the autophagy—lysosome system, and in particular chaperone-mediated autophagy, is associated with elevation of alpha-synuclein protein levels Alvarez-Erviti et al.