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Understanding the Noxious cause of Lou Gehrig's disease
September 14, 2007There is no known cure for amyotrophic lateral sclerosis (ALS), often known as Lou Gehrig disease and motor neuron disease. ALS is a progressive, fatal, neurodegenerative disease caused by the degeneration of nerves that control voluntary muscle movement. New evidence generated in mice by John Engelhardt and colleagues at Iowa University, Iowa City, has provided insight into the mechanisms responsible for certain forms of the disease and has identified potential targets for the development of drugs to treat individuals with these forms of ALS.
In some individuals, ALS is caused by a mutation in their SOD1 gene. Mice overexpressing this mutant gene (SOD1G93A mice) develop ALS-like disease. In this study, the authors found that the rate of disease progression could be dramatically slowed and survival markedly improved if SOD1G93A mice lacked expression of either Nox1 or Nox2, although the effects were more dramatic in the absence of Nox2. The Nox1 and Nox2 genes are on the X-chromosome and female mice lacking just one copy of either gene showed delayed disease onset, indicating that even a 50% decrease in expression of these proteins provided some protection. These data have led to the suggestion that developing drugs to inhibit the Nox pathway might be of benefit to individuals with ALS.
Journal of Clinical Investigation
ALS Current Events and ALS News RSSNew class of brain-protecting drugs emerging
Researchers have identified a compound that mimics one of the brain's own growth factors and can protect brain cells against damage in several animal models of neurological disease.
Gladstone scientists identify role of key protein in ALS and frontotemporal dementia
Scientists at the Gladstone Institute of Neurological Disease (GIND) have identified the reason a key protein plays a major role in two neurodegenerative diseases.
New ALS drug slips through telling 'phase II' clinical trials
A drug already used to treat symptoms of epilepsy has potential to slow the muscle weakening that comes with amyotrophic lateral sclerosis (ALS), scientists report after completing a Phase II clinical trial-an early, small-scale test to show if the drug works and continues to be safe.
Heavy metal paradox could point toward new therapy for Lou Gehrig's disease
New discoveries have been made about how an elevated level of lead, which is a neurotoxic heavy metal, can slow the progression of amyotrophic lateral sclerosis, or Lou Gehrig's disease - findings that could point the way to a new type of therapy.
Research sheds light on workings of anti-cancer drug
The copper sequestering drug tetrathiomolybdate (TM) has been shown in studies to be effective in the treatment of Wilson disease, a disease caused by an overload of copper, and certain metastatic cancers.
New understanding about mechanism for cell death after stroke leads to possible therapy
Scientists at the Brain Research Centre, a partnership of the University of British Columbia Faculty of Medicine and Vancouver Coastal Health Research Institute, have uncovered new information about the mechanism by which brain cells die following a stroke, as well as a possible way to mitigate that damage.
Schizophrenia gene's role may be broader, more potent, than thought
UCSF scientists studying nerve cells in fruit flies have uncovered a new function for a gene whose human equivalent may play a critical role in schizophrenia.
Compound shows potential for slowing progression of ALS
A chemical cousin of a drug currently used to treat sepsis dramatically slows the progression of amyotrophic lateral sclerosis, better known as ALS or Lou Gehrig's disease, in mice.
Dysfunctional protein dynamics behind neurological disease?
Researchers at Lund University, Sweden, have taken a snapshot of proteins changing shape, sticking together and creating structures that are believed to trigger deadly processes in the nervous system.
New guidelines identify best treatments to help ALS patients live longer, easier
New guidelines from the American Academy of Neurology identify the most effective treatments for amyotrophic lateral sclerosis (ALS), often called Lou Gehrig's disease. The guidelines are published in the October 13, 2009, issue of Neurology®, the medical journal of the American Academy of Neurology.
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