DNA Damage in ALS

October 19, 2013

BY AMY MADSEN ON WED, 2013-10-16 05:00

An MDA-supported team of researchers has linked ALS-causing mutations in the FUS gene to impaired DNA repair — a known cause of neurodegeneration.

A failure to repair damaged DNA appears to play an important role in amyotrophic lateral sclerosis (ALS), an MDA-supported team of researchers reports.

DNA, the molecule that encodes the “genetic blueprint” for life, often sustains damage, but various mechanisms employed by the cell typically repair it right away. When the cell’s DNA repair capabilities are impaired, however, it leads to the progressive loss of structure or function of nerve cells (neurodegeneration) that is seen in ALS and other diseases with neurological components.

Li-Huei Tsai at the Massachusetts Institute of Technology in Cambridge, Mass., and colleagues, showed that ALS-causing mutations in the FUS gene impair the DNA damage response in motor neurons, the muscle-controlling nerve cells that die in ALS.

The findings link DNA damage with ALS and suggest that therapies designed to boost the DNA repair process in motor neurons may be effective treatments for the disease.

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At Home in the ALS House

September 2, 2011

The future is now for 10 people living with ALS at this state-of-the-art residence overlooking Boston harbor.

Leonard Florence Center for Living

The Leonard Florence Center for Living is a state-of-the-art long-term care facility that’s currently the only Green House in the nation with a residence designed specifically for people with ALS. The ALS residence is named for landscape architect Steve Saling, who has ALS and contributed to the design and construction of the building.

To learn more about this fantastic facility and the inspiring man who helped to make it a reality, click here for the rest of the article.

MDA has awarded 10 grants totaling nearly $3.5 million to fund research projects focused on uncovering the causes of and developing therapies for ALS.

The new grants went to investigators at labs in the United States, Canada and Israel.

MDA’s Board of Directors met in Los Angeles July 16, where it reviewed and approved the new grants based on recommendations from the Association’s Scientific and Medical Advisory Committees (SAC and MAC). Grants are scored and recommended for approval based on the capabilities of the applicant, the scientific merit of the project, and the proposal’s relevance to developing treatments for the diseases in MDA’s program. The effective start date for the grants was July 1, 2010.

Click here to read the entire article.

With the holidays just around the corner and air travel likely for many families, now is the opportune time for people with disabilities to carefully plan their sojourns.

Learn from others

“Traveling with a disability is not easy, but with some extra preplanning and research, it is completely doable, and well worth it,” says Mike Bougher, 46, of Benicia, Calif., who received an ALS diagnosis in 1998.

In the travel tips section of his website, Bougher addresses air travel with a power wheelchair or ventilator, and even discusses “Wheelchairs in the Rainforests.”

Bougher provides comprehensive details about his travel preparations. For example, he and his wife Jen have wheelchair disassembly down to a fine art for commercial air travel. For the benefit of baggage handlers, the couple’s preparations include attaching two brightly colored laminated signs showing how to set and release the chair’s brakes.

Others with ALS have recounted their travel experiences, both good and bad, and how they made the experience more bearable, on Flyer Talk Forums/ALS Travel Advice. There you’ll find practical advice, such as to bring a blanket and extra socks to keep a person with ALS warm while flying. Noted one contributor, “[The] most important thing to remember is to help preserve his dignity. ALS is a disease of the body, not the mind.”

Click here to read the entire article.

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