Montreal Neurological Institute and Department of
Human Genetics, McGill University, Montreal, Canada
.eric@ericpc.mni.mcgill.ca
The inability to construct animal models of human diseases caused
by mutated mitochondrial DNA (mtDNA) has been a major obstacle to investigating
pathogenetic mechanisms associated with specific mtDNA mutations. Mice carrying
a large-scale deletion in mtDNA have now been produced. They display some
of the key features of the human disorder, but there are surprising differences.
