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Project

Exploring deranged calcium signaling in Wolfram syndrome: a role for anti-apoptotic Bcl-2? (2020-J1160630-214966)

Wolfram syndrome is a rare autosomal recessive genetic disorder, characterized by childhood-onset diabetes mellius, atrophy of the optic nerve and other complications. Mutations in two causative genes, WFS1 (majority of cases) and CISD2 (minority of cases) have been described as major disease caused. Currnently no treatment for Wolfram syndrome is available, resulting in a low life expectany (about 30 years). In Wolfram syndrome, intercellular Ca2+ release channels appear to be dysregulated, resulting in deranged Ca2+ dynamics and cellular stress. Important modulators of these channels are anti-apoptotic Bcl-2 proteins. By exploiting our insights in the cell biological functions of anti-apoptotic Bcl-2 proteins, we aim to examine whether Bcl-2 and Bcl-2-derived tools can normalize Ca2+ signaliing in Wolfram syndrome and suppress disease outcomes.

Date:1 May 2020 →  31 Dec 2020
Keywords:Wolfram-syndrome, Bcl-2 proteins
Disciplines:Cell signalling