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Advances in Disease Modeling for ALS and FTD Workshop

September 28, 2010

The ALS Association and the National Institute of Neurological Disorders and Stroke (NINDS) are proud to sponsor a workshop that will focus on the strategies used to generate Amyotrophic Lateral Sclerosis (ALS) and Frontotemporal lobar degeneration (FTLD) disease models and the lessons learned from the pathological, biochemical, molecular and behavioral analysis of these model systems.

For the past 15 years, investigators have relied on the use of mutations in SOD1 to model this motor neuron disease; yet, recent advances in the genetics of ALS provide opportunities to develop new animal models to study disease pathophysiology and to identify new directions for therapeutic development.  Investigators have utilized mutations in genes such as FUS/TLS and TARDBP to develop models of disease in a spectrum of diverse organisms including yeast, worms, flies, zebrafish and rodents.

FTLD represents a group of degenerative brain disorders characterized by progressive damage to neurons in the anterior temporal and/or frontal lobes of the brain.  Recent genetic advances have demonstrated that mutations in TARDBP, which encodes the trans-active response DNA-binding protein 43 (TDP-43) are also associated with FTLD.  The identification of TDP-43 protein pathology in both sporadic ALS and in the most common pathological subtype of FTLD (frontotemporal lobar degeneration with ubquitinated inclusions) further suggests a spectrum of diseases encompassing classic motor neuron disease at one end and FTLD at the other.

The workshop will be held on Friday, November 12 from 8 a.m. to 5 p.m., during the 2010 Society for Neuroscience Conference, at the Manchester Grand Hyatt hotel in San Diego, CA.  Investigators in the fields of ALS and FTLD are invited to participate in this one day workshop.  To register visit, http://guest.cvent.com/d/ndqv33/4W; the registration deadline is October 29.

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