Research Grant Award

Ilya Bezprozvanny, PhD

The University of Texas Southwestern Medical Center at Dallas
Deranged Calcium Signaling in SCA 3 Neurons

Spinocerebellar ataxia type 3 (SCA3) is a fatal neurodegenerative disorder. SCA is caused by mutation in protein ataxin-3. We discovered that mutated ataxin-3 binds to and activates the type 1 inositol (1, 4, 5)-trisphosphate receptor (InsP3R1)-neuronal intracellular calcium (Ca2+) release channel. In this grant I propose experiments with SCA 3 mouse model aimed to determine the importance of InsP3R1 association with mutated ataxin-3 for SCA 3 pathology.