Researchers Establish Mouse Model for Oxidative Stress

Their study was published in the September 26, 2002, issue of Nature.

Investigators from the Jackson Laboratory (Bar Harbor, ME, USA) worked with harlequin (Hq) mutant mice, a strain that suffers from progressive degeneration of terminally differentiated cerebellar and retinal neurons. These mice have a mutation in the apoptosis-inducing factor (AIf) gene that causes a severe reduction in AIF production. The AIF protein serves as a scavenger of free radicals in certain brain and retinal neurons. The low level of AIF protein causes defects in DNA replication that result in cell death and degeneration of the nervous system. Neurons damaged by oxidative stress in both the cerebellum and retina of Hq mutant mice re-enter the cell cycle before undergoing apoptosis.

The authors assert that the harlequin mouse provides the first model for studying the role of oxidative stress on aberrant cell cycle reentry and subsequent death of neurons.



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