26
September 2002
Neurodegenerative disease: Cycling to destruction
Harlequin mutant mice are a model for late-onset neurodegenerative disease, characterized by progressive degeneration of cerebellar and retinal neurons. The precise mutation has now been pinpointed as an insertion in the apoptosis-inducing factor gene (Aif). Disruption of Aif leads to oxidative stress and eventual neuron death. Prior to death the cerebellar granule cells and retinal ganglion cells re-enter the cell cycle, an emerging hypothesis for Alzheimer's disease. Cell cycle misregulation may be a common underlying mechanism for neurodegenerative diseases, and is a candidate target for therapies.
The harlequin mouse mutation downregulates apoptosis-inducing
factor
JEFFREY A. KLEIN, CHANTAL M. LONGO-GUESS,
MARLIES P. ROSSMANN, KEVIN L. SEBURN, RONALD E. HURD, WAYNE N. FRANKEL, RODERICK
T. BRONSON & SUSAN L. ACKERMAN
Nature 419, 367–374
(2002); doi:10.1038/nature01034
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