SEMINAR @ SISSA, SBP, dr. F. Feiguin - Oct. 12, at 15.00 hrs.

[Barbara Corzani]

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STATISTICAL AND BIOLOGICAL PHYSICS SEMINAR
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Dott. Fabian Feiguin
(ICGEB)

Title:
"Protein aggregation and neurodegeneration: Loss or gain of function effect?"

Mon 12 Oct. 2009 @ 03.00 p.m.

SISSA - Basement floor - room A
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Abstract:

Intracellular aggregation of the highly conserved ribonucleoprotein TDP-43 was recently associated to several neurodegenerative diseases including amyotrophic lateral sclerosis (ALS), a late-onset disorder that affects predominantly motoneurons. However, the function of TDP-43 in vivo is unknown and a possible direct role in neurodegeneration remains speculative. We found that flies lacking Drosophila TDP-43 presented deficient locomotive behaviors, reduced life span and anatomical defects at the neuromuscular junctions. These phenotypes were rescued by expression of the human protein in a restricted group of neurons including motoneurons. Our results demonstrate the role of this protein in vivo and suggest an alternative explanation to ALS pathogenesis that may be more due to the lack of TDP 43 function than to the toxicity of the aggregates.