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.
Original language | English |
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Pages (from-to) | 1043-1045 |
Number of pages | 3 |
Journal | Autophagy |
Volume | 5 |
Issue number | 7 |
DOIs | |
Publication status | Published - Oct 1 2009 |
Externally published | Yes |
Keywords
- Autophagy
- Cisd2
- Knockout mice
- Mitochondria
- Premature aging
ASJC Scopus subject areas
- Molecular Biology
- Cell Biology