Authors: Chen YF; Kao CH; Kirby R; Tsai TF
Abstract: CISD2, the causative gene for Wolfram syndrome 2 (WFS2), is a previously uncharacterized novel gene. Using a mouse genetic approach, this work demonstrated for the first time that Cisd2 is involved in mammalian life span control. Cisd2 deficiency in mice leads to mitochondrial breakdown and dysfunction; this is accompanied by cell death with autophagic features and these events precede the two earliest manifestations of nerve and muscle degeneration. Together, they lead to a panel of phenotypic features suggestive of premature aging. This work effectively links Cisd2 gene function, mitochondrial integrity and aging in mammals.
Keywords: Animals; Carrier Proteins/genetics/*metabolism; Cell Death/physiology; Female; Humans; *Life Expectancy; Mice; Mitochondria/*metabolism; Mitochondrial Proteins/genetics/metabolism; Nerve Tissue Proteins/genetics/*metabolism; Wolfram Syndrome/metabolism
Journal: Autophagy Volume: 5 Issue: 7 Pages: 1043-5 Date: Sept. 1, 2009 PMID: 19717971 |
Chen YF, Kao CH, Kirby R, Tsai TF (2009) Cisd2 mediates mitochondrial integrity and life span in mammals. Autophagy 5: 1043-5.
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