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Neuron. 2003 Jul 17;39(2):195-7.

Intracellular Ca2+ signaling and human disease: the hunt begins with Huntington's.

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Department of Pharmacology and Cellular and Molecular Physiology, Yale University School of Medicine, New Haven, CT, USA.


Huntingtin, a protein altered by polyglutamine expansion in Huntington's disease (Httexp), forms a signaling complex with the InsP3R, an intracellular calcium channel, and Htt-associated protein 1A (HAP1A). The addition of Httexp increases the InsP3R sensitivity to InsP3, which subsequently makes neurons hyperresponsive to stimulation and presumably more prone to neurodegenerative processes.

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