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12-18-2013, 07:58 AM | #1 | |||
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http://www.ascb.org/ascbpost/index.p...nson-s-disease
...In Parkinson's, it is the death of specific cells in the brain, dubbed dopaminergic cells, that leads to development of the disease. Mia Horowitz and Gali Maor of Tel Aviv University and colleagues hypothesized that this cell death cascade is the link to Parkinson's disease. They set out to make a laboratory model of carriers of GD mutations to test their idea using Drosophila melanogaster, the easily re-engineered fruit fly. They mutated the GD enzyme, glucocerebrosidase, in a subset of neurons in fruit flies. With defective glucocerebrosidases, these flies began losing their dopamine-generating neurons, a classic symptom of Parkinson's disease. Also, the Drosophila had reduced climbing ability, the fly equivalent of Parkinsonian hypokinesis, the characteristic movement "freeze-up." This is the first animal model in which carriers of GD mutations have demonstrated Parkinson's-like symptoms, says Horowitz. The fly model thus lends support to the idea that GD and Parkinson's disease are related through the pathways triggered by accumulation of defective proteins, a cascade that leads to dopaminergic cell death.
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