September 2012
18 September 2017

Years 2-4

1 October 2013 - 30 September 2016
ED 158
Université Pierre et Marie Curie

Characterization of dynein/dynactin mutations associated withamyotrophic lateral sclerosis and their effect on axonal transport andneuromuscular junction formation.

DEL BENE Filippo

Year 1

1 October 2012 - 1 June 2013
UEI (Unité d'enseignement isolé)
Université Pierre et Marie Curie

Lab rot 1: Including the SMN gene and protein in a pre-established neurodegenerative pathway using the zebrafish model - KABASHI Edor.  

Lab rot 2In vivo imaging of axonal transport (vesicles and organelles) and mRNA transport in a zebrafish dynactin mutant transgenic line.

DEL BENE Filippo

Biographie / Publications

Auer TO, Xiao T, Bercier V, Gebhardt C, Duroure K, Concordet JP, Wyart C, Suster M, Kawakami K, Wittbrodt J, Baier H, Del Bene F. Deletion of a kinesin I motor unmasks a mechanism of homeostatic branching control by neurotrophin-3.Elife. 2015 Jun 15;4. doi: 10.7554/eLife.05061.PMID: 26076409

Kabashi E, El Oussini H, Bercier V, Gros-Louis F, Valdmanis PN, McDearmid J, Mejier IA, Dion PA, Dupre N, Hollinger D, Sinniger J, Dirrig-Grosch S, Camu W, Meininger V, Loeffler JP, René F, Drapeau P, Rouleau GA, Dupuis L. Investigating the contribution of VAPB/ALS8 loss of function in amyotrophic lateral sclerosis. Hum Mol Genet. 2013 Jun 15;22(12):2350-60. doi: 10.1093/hmg/ddt080. Epub 2013 Feb 26. PMID: 23446633

Bercier V, Brustein E, Liao M, Dion PA, Lafrenière RG, Rouleau GA, Drapeau P. WNK1/HSN2 mutation in human peripheral neuropathy deregulates KCC2 expression and posterior lateral line development in zebrafish (Danio rerio). PLoS Genet. 2013;9(1):e1003124. doi: 10.1371/journal.pgen.1003124. Epub 2013 Jan 3. PMID: 23300475

Bercier V.WNK1/HSN2 isoform and the regulation of KCC2 activity.Rare Dis. 2013 Sep 19;1:e26537. doi: 10.4161/rdis.26537. eCollection 2013. PMID: 25003007

Kabashi E, Bercier V, Lissouba A, Liao M, Brustein E, Rouleau GA, Drapeau P. FUS and TARDBP but not SOD1 interact in genetic models of amyotrophic lateral sclerosis. PLoS Genet. 2011 Aug;7(8):e1002214. doi: 10.1371/journal.pgen.1002214. Epub 2011 Aug 4.PMID: 21829392

Kabashi E, Lin L, Tradewell ML, Dion PA, Bercier V, Bourgouin P, Rochefort D, Bel Hadj S, Durham HD, Vande Velde C, Rouleau GA, Drapeau P. Gain and loss of function of ALS-related mutations of TARDBP (TDP-43) cause motor deficits in vivo. Hum Mol Genet. 2010 Feb 15;19(4):671-83. doi: 10.1093/hmg/ddp534. Epub 2009 Dec 3. Erratum in: Hum Mol Genet. 2010 Aug 1;19(15):3102. PMID: 19959528


The optic tectum has emerged as a tractable visuomotor transformer, in which anatomical and functional studies can allow a better understanding of how behavior is controlled by neuronal circuits. We...