INT97861
From wiki-pain
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Sentences Mentioned In
Key: | Protein | Mutation | Event | Anatomy | Negation | Speculation | Pain term | Disease term |
Several animal studies also showed that depletion of GSL could be lethal or lead to neurological disturbance, and substrate reduction therapy should be aimed to reduce biosynthesis of GSL to a degree that can be tolerated by cells (Radin 1996). | |||||||||||||||
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These results are consistent with our hypothesis and with GSLs being centrally involved in the pathogenesis of NPC disease, and they suggest that drugs inhibiting GSL synthesis could have a similar ameliorating effect on the human disorder. | |||||||||||||||
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The inhibition of GSL synthesis by miglustat has been demonstrated to reduce pathological intracellular lipid storage, improve fluid-phase endosomal uptake and normalize lipid transport in peripheral blood B lymphocytes of NP-C patients.44 This leads to a reduction of the potentially neurotoxic accumulation of gangliosides GM2 and GM3, lactosylceramide and glucosylceramide, and may prevent further neuronal damage. | |||||||||||||||
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