Article
The stimulation of adenosine A2A receptors ameliorates the pathological phenotype of fibroblasts from Niemann-Pick type C patients.
The Journal of neuroscience : the official journal of the Society for Neuroscience - 25 Sept 2013
Visentin Sergio, De Nuccio Chiara, Bernardo Antonietta, Pepponi Rita, Ferrante Antonella, Minghetti Luisa, Popoli Patrizia
Abstract excerpt
Niemann-Pick type C1 (NPC1) disease is a rare neurovisceral disorder characterized by intracellular accumulation of unesterified cholesterol, sphingolipids, and other lipids in the lysosomal compartment. A deregulation of lysosomal calcium has been identified as one of the earliest steps of the degenerative process. Since adenosine A2A receptors (A2ARs) control lysosome trafficking and pH, which closely regulates...
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