Calpain inhibitor and ibudilast rescue β cell functions in a cellular model of Wolfram syndrome | Zendy

Lien D. Nguyen | Zendy; Tom T. Fischer | Zendy; Damien Abreu | Zendy; Alfredo Arroyo | Zendy; Fumihiko Urano | Zendy; Barbara E. Ehrlich | Zendy

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Calpain inhibitor and ibudilast rescue β cell functions in a cellular model of Wolfram syndrome

Author(s) -

Lien D. Nguyen,

Tom T. Fischer,

Damien Abreu,

Alfredo Arroyo,

Fumihiko Urano,

Barbara E. Ehrlich

Publication year - 2020

Publication title -

proceedings of the national academy of sciences

Language(s) - English

Resource type - Journals

eISSN - 1091-6490

pISSN - 0027-8424

DOI - 10.1073/pnas.2007136117

Subject(s) - wolfram syndrome , calpain , diabetes insipidus , neurodegeneration , atrophy , diabetes mellitus , medicine , regulator , disease , neuroscience , immunology , endocrinology , biology , gene , genetics , biochemistry , enzyme

Significance Wolfram syndrome is a rare multisystem disease characterized by diabetes insipidus, diabetes mellitus, optic nerve atrophy, and deafness (DIDMOAD). It is primarily caused by mutations in the Wolfram syndrome 1 gene,WFS1 . As a monogenetic disorder, Wolfram syndrome is a model for diabetes and neurodegeneration. There is no effective treatment for this invariably fatal disease. Here we characterize WFS1 as a regulator of calcium homeostasis and subsequently target calcium signaling to reverse deficits in a cellular model of Wolfram syndrome.

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