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Controversy “Are the molecular mechanisms of toxicity of C9orf72 caused by a loss-of-function?” (Yes/No) – June 1st, Strasbourg

June 1 @ 09:00 - 12:00



June 1
09:00 - 12:00
Event Category:


IGBMC 1 Rue Laurent Fries
Illkirch Graffenstaden, 67 France


Jose-Luis Gonzalez De Aguilar, Luc Dupuis & Pascale Piguet

This event will take place on June 1st in Illkirch, F.


The identification of a hexanucleotide repeat expansion mutation in a non-coding region of the gene on human chromosome 9 open reading frame 72 (C9orf72) has recently revolutionized the research on neurodegenerative diseases. C9orf72 mutation is the most common cause of hereditary amyotrophic lateral sclerosis (ALS), which is also the most common motor neuron disease in the adult. Abnormal C9orf72 expansion is also present in familial cases of frontotemporal dementia (FTD), which is frequently associated with ALS. At present, the way by which C9orf72 mutation triggers neurodegeneration remains unclear, and several mechanisms have been proposed based on either a loss or a gain of function. These mechanisms include the decrease in the amount of functional protein, the accumulation in RNA foci of aberrant forms of C9orf72 RNA, and the aggregation of dipeptide repeat proteins derived from an unusual translation process.
This controversy debate will expose arguments in support or in opposition to each of the postulated pathways. It will provide insight into the cellular and molecular aspects of such intriguing mechanisms of cell death. Discussions will draw the attention of not only experts on ALS/FTD research but also researchers in the field of neurodegeneration and beyond.


09.00 – 09.10 Presentation of the speakers (Jose-Luis Gonzalez De Aguilar, UMR_S INSERM 1118, Strasbourg, F)

09.10 – 09.30 Molecular mechanisms of toxicity of C9orf72: An Introduction (Luc Dupuis, UMR_S INSERM 1118, Strasbourg, F)

09.30 – 10.15 Arguments in Favour/Against a loss-of-function mechanism (Ludo van den Bosch, Leuven, B / Edor Kabashi, Paris, F / Clotilde Lagier-Tourenne, Charlestown, MA – 15 min each)

10.15-10.30 Coffee break

10.30-11.45 The Debate (Ludo van den Bosch, Leuven, B / Edor Kabashi, Paris, F / Clotilde Lagier-Tourenne, Charlestown, MA)

11.45 – 12.00 Conclusion (Luc Dupuis, UMR_S INSERM 1118, Strasbourg, F & Nicolas Charlet-Berguerand, IGBMC, Illkirch, F)


Download the poster/program: here



see below



This controversy debate will take place at IGBMC, 1 Rue Laurent Fries, Illkirch Graffenstaden.

Many thanks to the IGBMC for hosting this event.


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