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.

Related Organizations

Yale University
United States

Keywords

Huntington Disease, Animals, Humans, Calcium, Calcium Signaling, Signal Transduction

7 Research products, page 1 of 1

Huntingtin and Huntingtin-Associated Protein 1 Influence Neuronal Calcium Signaling Mediated by Inositol-(1,4,5) Triphosphate Receptor Type 1
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Intracellular Ca2+ signaling and human disease: the hunt begins with Huntington's.

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

7 Research products, page 1 of 1

Huntingtin and Huntingtin-Associated Protein 1 Influence Neuronal Calcium Signaling Mediated by Inositol-(1,4,5) Triphosphate Receptor Type 1

Intracellular Ca2+ Signaling and Human Disease

HAP1 facilitates effects of mutant huntingtin on inositol 1,4,5‐trisphosphate‐induced Ca2+ release in primary culture of striatal medium spiny neurons

Deranged neuronal calcium signaling and Huntington disease

Full length mutant huntingtin is required for altered Ca2+ signaling and apoptosis of striatal neurons in the YAC mouse model of Huntington's disease

HAP1 facilitates effects of mutant huntingtin on inositol 1,4,5-trisphosphate-induced Ca release in primary culture of striatal medium spiny neurons.

Inositol 1,4,5-tripshosphate receptor, calcium signalling and huntington’s disease

HAP1 facilitates effects of mutant huntingtin on inositol 1,4,5‐trisphosphate‐induced Ca²⁺ release in primary culture of striatal medium spiny neurons