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  • Altered receptors and Huntington

    images46Huntington’s disease (HD) is an inherited neurodegenerative disorder caused by a CAG triplet expansion, enabling long polyglutamine sequence in the huntingtin protein. The disease appears in about 40 years and presents with involuntary movements, rigidity and personality disorders and leads to death after 15-20 years of suffering. There is no known pathogenic mechanism or treatment for HD. A key advance in this study was that an American group reported that inhibitors of ATP synthesis reduced neuronal death in models of HD in Drosophila and C. elegans (somewhat unexpected because an inhibition of energy metabolism had been described in patients and mice). Simultaneously, a Korean group found that extracellular ATP induces neuronal cell death by activation of P2X7 receptor, an ion channel permeable to Ca2 +, which modulates neurotransmitter release from the terminal presynaptic neurons and the release of cytokines in microglia.

    This study conducted by Joseph J. Luke Center for Molecular Biology ‘Severo Ochoa’ is proposed to determine whether calcium permeability contributes to synaptic alterations and neuronal apoptosis described in HD. An increase in P2X7 receptor expression in animal models, plus an altered permeability to calcium-mediated P2X7 both presynaptically, as in neuronal cell bodies. The neurons expressing mutant huntingtin were more susceptible to apoptosis by P2X7 receptor activation. Finally, in vivo administration of HD transgenic mice BBG antagonist prevented the neuronal apoptosis and attenuated body weight loss and motor coordination deficits of more advanced stages of work animal.Este model posits the P2X7 receptor as a therapeutic target in HD and other neurodegenerative diseases.

    Published on November 14, 2012 · Filed under: Bioscience; Tagged as: , , ,
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