02 3 / 2012

Via Scoop.it - ALS Lou Gehrig’s Disease
The first auction of signed Beastie Boy memorabilia is live- Bidding is open from March 1st to March 8th 2012.
Via us1.campaign-archive1.com

01 3 / 2012

Via Scoop.it - ALS Lou Gehrig’s Disease
The ALS Association and the Robert Packard Center for ALS Research at Johns Hopkinshave entered into a partnership to expedite the development of animal model systems to expand the knowledge about the C9ORF72 gene, which has been identified as the most common cause of inherited amyotrophic lateral sclerosis (ALS or Lou Gehrig’s Disease) and Frontotemporal dementia (FTD).“The Association is very pleased to partner with the Packard Center to expedite these important studies,” said ALS Association Chief Scientist Lucie Bruijn, Ph.D. “The Association along with the Packard Center have both invested significant funds into the identification of this new gene, and we are pleased to be able to work together to support the critical next steps to ensure that possible discoveries from these projects are translated as rapidly as possible into therapies for ALS. In October, 2011, a large expansion of a hexanucleotide GGGGCC repeat was discovered in the C9ORF72 gene, but how the expansion causes malfunction of the nerve cells in ALS and FTD remains unknown. It is thought that the messenger RNA (mRNA) derived from this large repeat aberrantly accumulates. This scenario is reminiscent of what is known in other diseases caused by expanded repeats, especially myotonic dystrophy. Building on that example and in partnership with Isis Pharmaceuticals, the Cleveland Laboratory in San Diego, Calif., has designed a gene silencing approach to develop a drug called an antisense oligonucleotide (ASO) that will selectively destroy the ALS-causing mRNA with the expanded repeat. Essential for drug development is a mouse model expressing the expanded human C9ORF72 mRNA. The investigators will build these models and use them to validate efficacy of the ASO drug.
Via alscenter.org

01 3 / 2012

Via Scoop.it - ALS Lou Gehrig’s Disease
It is an incredible story of courage and home. Cougar football great and Gonzaga Prep grad Steve Gleason is battling ALS - Lou Gehrig’s Disease - which has no known cure. But, he’s not waiting around for the disease to kill him. He’s living life and helping others diagnosed with the same disease.
Via kxly.com

29 2 / 2012

Via Scoop.it - ALS Lou Gehrig’s Disease
In science, refuting a hypothesis can be as significant as proving one, all the more so in research aimed at elucidating how diseases proceed with a view toward preventing, treating, or curing them. Such a discovery can save scientists from spending precious years of effort exploring a dead end. In a study published in the Proceedings of the National Academy of Sciences, Munich-based researchers refute a widely accepted hypothesis about a causative step in neurodegenerative conditions. These results deal specifically with animal models of human amyotrophic lateral sclerosis (ALS, aka Lou Gehrig’s disease) but also raise questions for research on other neurodegenerative diseases, such as Alzheimer’s or Huntington’s disease.
Via eurekalert.org

29 2 / 2012

Via Scoop.it - ALS Lou Gehrig’s Disease
The quest for treatments for motor neurone disease, spinal cord injury and strokes could be helped by new research that shows how key cells are produced. Scientists at the University of Edinburgh have been able to manipulate the production of motor neurones – which control all muscle activity – in zebrafish. Zebrafish are important in helping scientists understand how motor neurones are produced, because unlike mammals, they are able to create new motor neurones as adults. Humans can generate motor neurones during embryonic development but lose the ability to generate these cells, which are important for speaking, walking and breathing, after birth. This means that the body is unable to replace these cells if they become damaged as a result of motor neurone disease, stroke or spinal cord injury.
Via eurekalert.org

29 2 / 2012

Via Scoop.it - ALS Lou Gehrig’s Disease
The American Academy of Neurology Foundation and The ALS Association are announcing the creation of the Richard Olney Clinician Scientist Development Award in ALS, named after Richard K. Olney, M.D., a leading neurologist and pioneer in clinical ALS research who died late last month of ALS, also known as amyotrophic lateral sclerosis, or Lou Gehrig’s Disease. We came together to create this research fellowship in record time as a tribute to Dr. Olney, not only as a courageous person, but one whose pioneering ALS research improved the lives of many people diagnosed with this dreadful disease that gradually robs the body of its ability to use its muscles,” said John Mazziotta, M.D., Ph.D., Chair of the American Academy of Neurology Foundation’s Board of Trustees and Professor and Chair of UCLA’s Brain Mapping Center in Los Angeles. Applications for the $240,000 research prize will be accepted through October 1, 2012.
Via sfgate.com

29 2 / 2012

Via Scoop.it - ALS Lou Gehrig’s Disease
Animals regulate body weight using an exquisitely tuned system. If the body needs fuel, the animal can go in search of food. Any extra fuel is stored as fat, to be used when energy stores run low. Through a complex series of checks and balances, animals (including humans) generally maintain a stable body weight, where energy consumed equals energy used. In ALS patients, however, something throws off this balance. Besides the symptoms stemming from deteriorating motor neurons, such as difficulties walking, talking, breathing, and even swallowing, ALS patients often lose weight. Although some of this weight loss is perhaps due to muscle wasting, scientists have also noticed that ALS patients often lose a significant amount of body fat. When Robert Kalb, a neuroscientist at the Children’s Hospital of Philadelphia, and his colleagues searched the research literature, however, they realized that no one really knew what caused weight to drop in ALS. Nor did scientists know whether the weight loss contributed to the progression of ALS. A series of experiments in tissue culture and in the model organism Caenorhabditis elegans showed that ALS patients have a metabolic abnormality that contributes to both weight loss and the progression of disease.
Via alscenter.org

29 2 / 2012

Via Scoop.it - ALS Lou Gehrig’s Disease
On February 9 and 10, ALS researchers from P2ALS and several different pharmaceutical companies gathered in New York as part of the semi-annual meetings of P2ALS. P2ALS was founded in 2010 as a joint effort between The Robert Packard Center for ALS Research and Project A.L.S. with the goal of finding new treatments for ALS. This collaboration established a highly cooperative network of scientists that share unpublished findings to facilitate rapid testing and verification of complex research questions posed by ALS. Initially, P2ALS focused on genetics, and motor neuron and glial biology— basic science that allowed the identification of pathogenic pathways in ALS and potential therapeutic targets. At this most recent meeting held in New York City, the headquarters of Project A.L.S., scientists from around the world shared their most recent research, which covered advances in motor neuron and astrocyte biology, the genetics of familial and sporadic ALS, including new avenues of study opened up by the discovery of the C9ORF72 gene, and recent progress in the use of stem cells in the study and treatment of ALS.
Via alscenter.org

27 2 / 2012

Via Scoop.it - ALS Lou Gehrig’s Disease
Mitochondria produce the energy needed to keep muscles healthy and moving. But in people with ALS, these power plants go out of service likely contributing to muscle atrophy and ultimately, paralysis. Scientists are developing treatments to supe up mitochondria in hopes to keep the energy flowing in the muscles and connecting nerves. One of these emerging medicines, Knopp Biosciences’ dexpramipexole now licensed by Biogen Idec, is gathering steam as a potential ALS treatment strategy. Reporting phase II results last fall, neurologists found that the drug slowed disease progression about 30%. Some researchers suspect however, that these mitochondrial-targeted medicines may need to do much more than boost energy production to grind ALS to a halt. Even operating at full steam, these power plants may be unable to provide enough energy to keep muscles working because they may not be in the right place to do their job. Scientists are now beginning to understand why these mitochondria might stray, suggesting new strategies to tackle the disease.
Via blogs.als.net

26 2 / 2012

Via Scoop.it - ALS Lou Gehrig’s Disease
Several neurodegenerative diseases - including Alzheimer’s and ALS (Lou Gehrig’s disease) - are caused when the body’s own proteins fold incorrectly, recruit and convert healthy proteins to the misfolded form, and aggregate in large clumps that gum up the works of the nervous system. “For Star Trek fans, this is like the Borg, [a fictional race of cyborgs that abduct and assimilate humans and other species],” says Steven Plotkin, a biophysicist at the University of British Columbia in Vancouver who studies the process of protein misfolding. Plotkin’s team has developed an algorithm that can predict which regions of a protein are prone to exposure upon misfolding, and how mutations in the protein and changes in the cellular environment might affect the stability of these vulnerable regions. These predictions help scientists gain a better understanding of protein dynamics, and may one day help in developing treatments to effectively combat currently incurable neurodegenerative diseases. The team will present its findings at the 56th Annual Meeting of the Biophysical Society (BPS), held Feb. 25-29 in San Diego, Calif.
Via medicalnewstoday.com