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Lou Gehring's Disease

posted 6 Nov 2013, 08:40 by Mpelembe Admin   [ updated 6 Nov 2013, 08:40 ]

University of Manitoba researchers have uncovered a gene that may be driving the fatal neurodegenerative disease amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig’s disease.

The gene, known as TLS/FUS, is mutated in ALS patients. The mutant gene produces vast amounts of a faulty protein. Accumulation of the protein within cells is characteristic of the disease.

"Rather than turning off the production of a protein the cell knows is bad, it just keeps on plowing and plowing and plowing more, creating a worse situation," Geoff Hicks, director of theregenerative medicine program at the Winnipeg-based university, said in a CBC report.

Hicks’ team at the university found a way to manipulate the cells to stop making the mutant protein.

Although it may take many years to develop this finding into an intervention, it has the potential to extend the lifespan of ALS patients, Hicks said.

ALS, also known as Lou Gehrigs disease after the famous New York Yankees baseball player who retired from the sport after developing the disease in 1939, is the progressive degeneration of motor neurons that control voluntary muscle movement.

SOURCES:

National Institute of Neurological Disorder and Stroke

ALS Association

Winnipeg Free Press

Public Library of Science, Genetics

http://www.ninds.nih.gov/disorders/amyotrophiclateralsclerosis/detail_ALS.htm

http://www.alsa.org/about-als/what-is-als.html

http://www.winnipegfreepress.com/local/u-of-m-team-finds-a-key-to-als-230164611.html

http://www.plosgenetics.org/article/info%3Adoi%2F10.1371%2Fjournal.pgen.1003895

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