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Mouse Model of Lou Gehrig's Disease.
Ingenium Pharmaceuticals AG (Fraunhoferstrasse 13, 82152 Martinsried; Tel: +49 89 8565-2300, Fax +49 89 8565-2305; Website: www.ingenium-ag.com) and a coalition of international research organizations have reported in Science results concerning the genetic and molecular basis for motor neuron disease (MND), which includes amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease. The research explains a key pathogenetic mechanism of motor neuron degeneration, potentially opening new therapeutic avenues for treating these diseases. The research was conducted by Ingenium and several European academic collaborators.
The group showed that missense point mutations in the cytoplasmic dynein heavy chain result in progressive motor neuron degeneration in heterozygous mice. In mice that are homozygous for mutation, degeneration is accompanied by the formation of Lewy-like inclusion bodies, resembling the human pathology. The mutations interfere with neuron-specific functions of dynein. Prior studies of this particular gene in knock-out animal models produced only embryonic lethalities.
By identifying two specific mutations in the same gene, the research group has produced a precise mammalian model of MND and described the pathogenetic link between specific gene mutations and ...
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- Magazine article from: Environmental Health Perspectives Phelps, Jerry December 1, 2006 700+ words
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- News wire article from: Charlotte Observer (Charlotte, NC ) April 25, 2007 700+ words
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