The CISD2 protein is a zinc finger protein that is targeted to the endoplasmic reticular membrane. The protein binds an iron/sulfur cluster, and is speculated to be involved in calcium homeostasis. Defects in the protein have been implicated in the development of Wolfram Syndrome 2, a condition characterized by diabetes mellitus, optic atrophy, and sensorineural hearing loss.
Although the exact role played by the CISD2 protein in the pathogenesis of Wolfram Syndrome is not known, the mechanism of action of the protein is very similar to Wolframin, another protein implicated in the same disease condition. Thus, researchers hypothesize that defects in the protein result in impaired calcium homeostasis, that cause defects in protein folding, specifically insulin folding, and thus lead to diabetes and hearing loss.