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Montreal scientists discover origins of rare neurological disease.
un sujet a posté bxlmontreal dans L'actualité
Lead team of international researchers. MONTREAL - An international team of researchers led by scientists from the Montreal Neurological Institute and Hospital at McGill University have discovered the origins of a rare neurological disease known as ARSACS first appears in children and exists almost exclusively in Quebec. The discovery, published in this week’s issue of the Proceedings of the National Academy of Sciences of the United States of America and singled out for special mention by the American Society for Cell Biology, revealed the disease is linked to a defect in the function of mitochondria, the energy-producing power plants of cells, which gives it a link to more common neurological diseases like Parkinson’s and ALS. This brings hope the extensive research done on those diseases will advance treatment of the rare disorder and further research in the more common diseases. “We think that by studying this disease we will not only bring treatment to those patients, but may also help to better understand how other neuro-degenerative diseases like Parkinson’s – and to a lesser degree, Alzheimer’s – function,” said Dr. Bernard Brais of the Neuro. Autosomal Recessive Spastic Ataxi of Charlevoix-Saguenay is so named because it was diagnosed among descendants of roughly 10,000 French settlers who emigrated to that region of New France. It was first recognized as a separate neurological disease in 1979. Victims show symptoms between the ages of 2 and 6 years old that include trouble walking and clumsiness. By their early 40s, sufferers must use wheelchairs and have trouble speaking. Most die in their 50s. The disease affects about 300 people in Quebec, and another 100 worldwide. In 2000, the gene that was mutated in patients was identified. All genes make proteins that carry out a certain function, but scientists didn’t know what the mutated proteins did. This year, a team of 30 scientists working at nine laboratories worldwide finally discovered that the mitochondria in neurons (cells that carry nerve impulses), particularly those found in the brain, were mutated and would shut down, leading to neurological degeneration. “You can’t even imagine what to do in terms of therapy until you know at the cellular level what’s going on,” said Dr. Peter McPherson of the Neuro. Scientists can now try different medications and chemical treatments to slow or cure the disease. Most importantly, because links were found to diseases like Parkinson’s and ALS that are also affected by mitochondrial defects, the multitude of research on those diseases, and the treatments used, can be applied to ARSACS. “For us, it definitely gives hope,” said Sonia Gobeil and Jean Groleau, whose two children were diagnosed in 2006. The Montreal couple have raised more than $2 million for research. Their children, now 6 and 8, are still fine but “we know where its going,” Gobeil said. Anything that can help to slow or cure the disease is key, the couple said. This week’s announcement was a huge step. Read more: http://www.montrealgazette.com/news/Montreal+scientists+discover+origins+rare+neurological+disease/6005135/story.html#ixzz1jkNPkYI5 -
Un fil dans lequel tous nous pourrons partager nos blagues. A thread in which everyone of us will be able to shares jokes with the others. Here's the first of (I hope) many : Better than a Flu Shot! Miss Beatrice, the church organist, was in her eighties and had never been married. She was admired for her sweetness and kindness to all. One afternoon the pastor came to call on her and she showed him into her quaint sitting room. She invited him to have a seat while she prepared tea. As he sat facing her old Hammond organ, the young minister noticed a cut-glass bowl sitting on top of it. The bowl was filled with water, and in the water floated, of all things, a condom! When she returned with tea and scones, they began to chat. The pastor tried to stifle his curiosity about the bowl of water and its strange floater, but soon it got the better of him and he could no longer resist .. 'Miss Beatrice', he said, 'I wonder if you would tell me about this?' pointing to the bowl. 'Oh, yes,' she replied, 'Isn't it wonderful? I was walking through the Park a few months ago and I found this little package on the ground. The directions said to place it on the organ, keep it wet and that it would prevent the spread of disease. Do you know I haven't had the flu all winter.'
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Montreal team makes HIV discovery Virus gets help from a cell protein. Finding is expected to help development of new class of drugs to combat the disease CHARLIE FIDELMAN, The Gazette Published: Saturday, July 14 Montreal researchers have identified a novel target that's an accomplice to HIV, the virus that causes AIDS. The virus doesn't work alone, but recruits a collaborator - a cell protein - in its mission to multiply and spread through the body, explained Eric Cohen, a professor of medicine at the UniversitE de MontrEal. Cohen and his team of researchers at the Institut de recherches cliniques de MontrEal yesterday published the findings in PLoS Pathogens, a peer- reviewed journal that is issued monthly by the Public Library of Science. Email to a friendPrinter friendly Font: * * * * Despite having transformed HIV/AIDS from a fatal disease to to a chronic one, scientists are still at a loss to explain how the human immunodeficiency virus causes illness and why it persists despite highly effective anti-retroviral drug therapy. The discovery by Cohen's team is expected to open the door to the development of a new class of drugs to combat the disease, Canada's top HIV experts stated yesterday. Human immunodeficiency virus type 1 (HIV-1) leads to AIDS by depleting essential immune cells called CD4+T lymphocytes in infected individuals. Key to this process is a small HIV protein, said Cohen, who identified the viral protein R (Vpr) a decade ago while at the Dana-Farber Cancer Institute, affiliated with Harvard University in Cambridge. Mass.. "The role of this protein, called E3 ubiquitin ligase complex, is likely to control the orderly division of cells," Cohen said. HIV uses this protein to weaken infected immune cells. The cells then stop dividing and die, he explained. Also, the protein helps sabotage immune cells so the virus can harness their resources for its own purposes - that is, to replicate and spread the infection. "The virus is creating an environment inside the cells where it can multiply better. Ultimately, the cells will die," said Cohen, who holds the Canada Research Chair in human retrovirology at the U de M. Although proud of his work and of the team effort in his laboratory, Cohen cautioned that new therapies will not be available for years to come. "As with all basic fundamental discoveries, it will not lead to new types of (drugs) for six or seven years," he said. But it's a very important finding, said Rafick-Pierre SEkaly, a U de M immunologist and AIDS expert who was not involved in this discovery. "We are always looking for new ways to neutralize the virus, so finding a new target is very appealing," SEkaly said. Cohen's work explains how the virus corrupts immune cells, said virologist Mark Wainberg, director of the McGill AIDS Centre at the Jewish General Hospital. Wainberg described Cohen's finding as "exactly the kind of discovery that will excite drug companies." cfidelman@ thegazette.canwest.com
