Cisd2 mediates mitochondrial integrity and life span in mammals.
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.
Chen
Yi-Fan
YF
Department of Life Sciences and Institute of Genome Sciences, National Yang-Ming University, Taipei, Taiwan.
Kao
Cheng-Heng
CH
Kirby
Ralph
R
Tsai
Ting-Fen
TF
D000818:Animals; D000071183:Autophagy-Related Proteins; D002352:Carrier Proteins / Q000235:genetics / Q000378:metabolism*; D016923:Cell Death / Q000502:physiology; D005260:Female; D006801:Humans; D008017:Life Expectancy; D051379:Mice; D008928:Mitochondria / Q000378:metabolism*; D024101:Mitochondrial Proteins / Q000235:genetics / Q000378:metabolism; D009419:Nerve Tissue Proteins / Q000235:genetics / Q000378:metabolism*; D014929:Wolfram Syndrome / Q000378:metabolism
D000071183:Autophagy-Related Proteins; D002352:Carrier Proteins; D024101:Mitochondrial Proteins; D009419:Nerve Tissue Proteins; C515811:Noxp70 protein, mouse