Minnesota state Sen. Gary Kubly dies of ALS

Via Scoop.it – ALS Lou Gehrig’s Disease
Minnesota Sen. Gary Kubly, DFL-Granite Falls, who had suffered from amyotrophic lateral sclerosis (ALS, or Lou Gehrig’s disease), died Friday, according to a message sent to legislators Saturday afternoon.Kubly, 68, had undergone a medical crisis Wednesday morning and was being treated at Regions Hospital in St. Paul. Gary was a dedicated public servant and an outstanding leader,” Gov. Mark Dayton said Saturday in a statement. “He lived his life with quiet strength and principled dignity, as evidenced by the remarkable courage with which he faced his final illness. He is an inspiration to all of us who knew and worked with him.” Kubly, a Lutheran pastor, was first diagnosed with the neurological disease in 2010. At the start of this year’s legislative session, Kubly had programmed his iPad to speak a message of thanks for him.
Via www.startribune.com

New documentary chronicles life of Bay Area musician with Lou Gehrig’s disease

Via Scoop.it – ALS Lou Gehrig’s Disease

A Richmond man who has defied the odds in music and medicine is the star of a new documentary premiering in San Jose.Jason Becker was on his way to becoming a rock star. But that didn’t happen. At age 20, Jason was diagnosed with ALS, also known as Lou Gehrig’s disease. He slowly lost the ability to control his body. Doctors gave him just three to five years to live. When ABC7 first met him in 1996, he had already proved the doctors wrong. Now he is 42 years old.And almost more incredible, during all those years, even though Jason can’t move or speak, he has continued composing spectacular music. His music has fans all over the world, from heavy metal guitar lovers to ballet companies.ABC7 talked director Jesse Vile about his film “Jason Becker: Not Dead Yet” and why he wanted to make a movie about Jason. “His story isn’t just the story of a rock star or a kid trying to realize his dreams, it’s really the story of family and love and triumph over adversity; those are all themes that are universal,” Vile said.
Via abclocal.go.com

Zebrafish study provides innovative ideas for new treatment strategies

Via Scoop.it – ALS Lou Gehrig’s Disease

A study on zebrafish has increased our understanding of how motor neurones work and has provided potential clues for the development of future treatments for MND. The study, led by Dr Catherina Becker from University of Edinburgh, showed that a unique motor neurone repair system found in zebrafish can be enhanced if a particular signal called Notch1 is stopped. The results were published in the 29 February edition of Journal of Neuroscience. Unlike us, zebrafish have the ability to regenerate motor neurones when they’re damaged. This means that zebrafish can provide scientists with an excellent tool to find out about motor neurone repair. A better understanding how zebrafish achieve neurone regeneration could provide clues to develop new treatment strategies for MND. From their studies, Dr Becker and colleagues found that a chemical signaling pathway called Notch1 is increased when the neurones are damaged in zebrafish. By over activating the Notch1 signal, they found that new motor neurones would not grow well, and would not grow in the same prolific numbers as normal. This means that an over active Notch1 signal can stop the regeneration of motor neurones that occurs in zebrafish.
Via mndresearch.wordpress.com

C9ORF72 Comes Into Focus

Via Scoop.it – ALS Lou Gehrig’s Disease
Neurologists may need to keep an eye out for cognitive and behavioral changes in people showing signs of ALS, according to a new study. The research team, led by Trinity College Dublin neurologist Orla Hardiman MD FRCP, found that 50% of people examined with the most common form of familial ALS identified to date also showed signs of frontotemporal lobar degeneration (FTLD). The brain disorder might result in difficulties in critical thinking, problem solving and making complex decisions. The study, which included 20 people with familial ALS harboring repeat expansions in the C9ORF72 gene, is the first to clinically describe this form of ALS. The study is one of three studies this month that confirms that repeat expansions in the C9ORF72 gene are the most common cause of inherited forms of ALS, ALS-FTLD and FTLD.
Via blogs.als.net

A Passion For The Rainforest Leads Botanist To Fight Human Diseases

Via Scoop.it – ALS Lou Gehrig’s Disease

A prominent botanist with a passion for preserving the rainforest and exploring medicines in native cultures is helping to make inroads into one of the most difficult problems in medical research: understanding and treating the neurological diseases Alzheimers, Parkinsons and amyotrophic lateral sclerosis, or ALS, better known as Lou Gehrig’s disease. The botanist is Paul Allan Cox, whose studies of plants and human diets on Guam a decade ago have led to one of the most promising approaches to understanding the diseases, which afflict millions worldwide. In 2003, Cox showed that a natural neurotoxin called BMAA becomes concentrated in the diets of certain Guananian villagers, who then develop some of the world’s highest rates of ALS. Since then, he and researchers around the world have found elevated levels of BMAA in the brains of Alzheimer’s and ALS patients in North America. They have identified a universal source of BMAA in blue-green algae called cyanobacteria that are found nearly everywhere. Now, they have taken early steps to explore the detailed biophysical role that BMAA may be playing in causing the failure of human motor neurons. Ever one to explore native cultures, Cox has even found an unusually healthy village on the Japanese island of Okinawa where exceptionally long-lived residents might offer clues to something neurologically protective in the diet. Cox discussed his findings at Livermore’s Bankhead Theater last week as part of the Rae Dorough Speaker’s Series.
Via www.independentnews.com

Actress, Gina Bellman, shares her experience of MND

Via Scoop.it – ALS Lou Gehrig’s Disease
Many of you will remember Gina for her roles in Dennis Potter’s ‘Blackeyes’, or as the hilarious Jane in the sitcom ‘Coupling’. Gina’s mother, Helen, has MND and Gina describes how the disease has impacted on the Bellman family.
Via sarahezekiel.blogspot.com

Beastie Boys Auction to fight ALS/Lou Gehrig’s disease

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

The ALS Association and the Packard Center Partner to Develop Animal Model Systems for Most Common Cause of Familial ALS

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 www.alscenter.org

Coming Home: The Steve Gleason Story

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 www.kxly.com