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Gene Therapy and Regulation , Vol. 2, No. 4, pp. 283 – 300 (2004) VSP 2004. Also available online - www.vsppub.com Xenomitochondrial embryonic stem cells and mice: modeling human mitochondrial biology and disease ∗ MATTHEW V. CANNON 1 , CARL A. PINKERT 1 , † and IAN A. TROUNCE 2 , † 1 University of Rochester Medical Center, Department of Pathology and Laboratory Medicine, Center for Aging and Developmental Biology, Rochester NY, USA 2 University of Melbourne, Centre for Neuroscience, Victoria, Australia Received 10 February 2005; revised 2 March 2005 Abstract —The characterization of mitochondrial diseases has proceeded rapidly since the first descriptions of mitochondrial DNA (mtDNA)-linked disease mutations appeared in the late 1980s. To elucidate mechanisms of a variety of mitochondrial disorders and disease, both in vitro and in vivo modeling systems have been exploited. To produce these models, numerous approaches have been undertaken due to the difficulty associated with targeted mutagenesis and directed modification of the mitochondrial genome. Currently available models of mitochondrial disease are discussed in this paper, including our xenomitochondrial mice. In this model, mitochondria from one donor species are transferred to another. By doing so, cells and animals were generated with varying
Gene Therapy and Regulation – Brill
Published: Jan 1, 2004
Keywords: MITOCHONDRIA; TRANSGENIC ANIMALS; RHODAMINE-6G; GENE TRANSFER; GENETIC ENGINEERING; EMBRYONIC STEM CELLS; HETEROPLASMY
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