By studying rodent models of the relatively rare inherited form of Lou Gehrigs disease and tissue samples from a patient with the condition, scientists have discovered the first evidence that damage to nerve cell powerhouses is directly responsible for these cells death. The findings appear in the July 9 issue of Neuron.
The research team from the University of California San Diego, Johns Hopkins and elsewhere discovered that dysfunctional proteins clog the transport system that brings vital substances into mitochondria, the tiny organelles that provide energy to cells. This mitochondrial damage occurs in muscle-controlling nerve cells, the researchers report, helping explain the selective nature of inherited amyotrophic lateral sclerosis (ALS), or Lou Gehrigs disease.
"Mitochondria dont look normal in motor neurons in animal models of ALS and in patients with ALS, but this is the first study that links ALS and a specific problem with the mitochondria," says study co-author Jeffrey Rothstein, M.D., Ph.D., professor of neurology and director of the Robert Packard Center for ALS Research at Johns Hopkins.
| newswise
Further information:
http://www.hopkinsmedicine.org
http://www.alscenter.org
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