Defects in the CISD2 (CDGSH iron sulfur domain 2) gene are a cause of the neurodegenerative disorder Wolfram syndrome 2. CISD2 is a zinc finger protein that localizes to the endoplasmic reticulum and mitochondria and binds an iron/sulfur cluster. CISD2 interacts with Bcl-2 and can be displaced by the BH3-only protein BIK and contributes to the regulation of BIK-initiated autophagy. CISD2 deficiency in mice causes mitochondrial breakdown accompanied by autophagic cell death as well as the development of premature aging phenotype.
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Protein Aliases: CDGSH iron sulfur domain-containing protein 2; CDGSH iron-sulfur domain-containing protein 2; Endoplasmic reticulum intermembrane small protein; Miner1; mitoNEET related 1; MitoNEET-related 1 protein; NAF-1; nervous system over-expressed protein 70; Nervous system overexpressed protein 70; Nutrient-deprivation autophagy factor-1; zinc finger, CDGSH-type domain 2
Gene Aliases: 1500009M05Rik; 1500026J14Rik; 1500031D15Rik; AI848398; B630006A20Rik; CDGSH2; CISD2; ERIS; Miner1; NAF-1; Noxp70; RGD1566242; WFS2; ZCD2