IGBMC : Institut de la Génétique et de la Biologie Moléculaire et Cellulaire

• Researchers

  Juliette GODIN

• Post-Doctoral Fellows

  Laure ASSELIN

  Efil BAYAM

  Hakima FLICI TEZKRATT

• PhD Students

  Jordi DEL POZO RODRIGUEZ

  José RIVERA ALVAREZ

• Engineers & Technicians

  Peggy TILLY

Translational medicine and neurogenetics

Regulation of cortical development in health and disease  

Cortical lamination follows an inside-out sequence of neuronal placement and maturation that arises from the sequential birth and orderly migration of projection neurons and GABAergic interneurons. Cerebral cortical neurogenesis involves a tight coordination of multiple cellular activities including neuronal specification, migration and differentiation. Disrupting the completion of one or several of these steps often cause cortical malformations that lead to severe learning disabilities, mental retardation and epilepsy. Remarkably, mutations in proteins associated with the centrosome, the major microtubule organizer in animal cells, have been linked to several diseases of brain development. Those neurodevelopmental disorders are thought to involve abnormal neuronal proliferation, migration, differentiation and/or maturation, suggesting crucial roles of the centrosome at those different neuronal developmental stages. Interestingly growing evidences suggest a high sensitivity of the brain to centrosomal abnormalities.

What makes developing brain more affected by centrosomal defects? Does the centrosome have a specific protein signature during cortical development? What is the physiological importance of this signature? To which extent and how any alteration of this cortical signature contribute to neurodevelopmental pathologies?

Answers to those questions would be critical to interpret the pathological mechanisms that participate in the onset and the progression of neurological disorders. Despite the identification of hundreds of centrosomal proteins, the underlying mechanisms by which this organelle coordinates these different steps of cortical development remain largely unknown.

The major goal of the team is to better characterize the centrosome-regulated cellular and molecular pathways that contribute to the cortical embryonic neurogenesis in heath and disease.

• Current projects

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• Publications

  • Mutations in the KIF21B kinesin gene cause neurodevelopmental disorders through imbalanced canonical motor activity.

    Asselin L(1)(2)(3)(4), Rivera Alvarez J(1)(2)(3)(4), Heide S(5)(6)(7), Bonnet CS(1)(2)(3)(4), Tilly P(1)(2)(3)(4), Vitet H(8), Weber C(1)(2)(3)(4), Bacino CA(9)(10), Baranaño K(11), Chassevent A(11), Dameron A(12), Faivre L(13)(14), Hanchard NA(9), Mahida S(15), McWalter K(12), Mignot C(5)(6)(7)(16), Nava C(5)(16), Rastetter A(16), Streff H(9)(10), Thauvin-Robinet C(13)(17), Weiss MM(18), Zapata G(10), Zwijnenburg PJG(18), Saudou F(8), Depienne C(1)(2)(3)(4)(16)(19), Golzio C(1)(2)(3)(4), Héron D(5)(6)(7), Godin JD(20)(21)(22)(23).

    Nat Commun May 15, 2020 ; 11:2441 .

  • TUBG1 missense variants underlying cortical malformations disrupt neuronal locomotion and microtubule dynamics but not neurogenesis

    Ekaterina L Ivanova 1 2 3 4 , Johan G Gilet 1 2 3 4 , Vadym Sulimenko 5 , Arnaud Duchon 1 2 3 4 , Gabrielle Rudolf 1 2 3 4 , Karen Runge 1 2 3 4 , Stephan C Collins 1 2 3 4 6 , Laure Asselin 1 2 3 4 , Loic Broix 1 2 3 4 , Nathalie Drouot 1 2 3 4 , Peggy Tilly 1 2 3 4 , Patrick Nusbaum 7 , Alexandre Vincent 1 2 4 , William Magnant 1 2 4 , Valerie Skory 1 2 3 4 , Marie-Christine Birling 8 , Guillaume Pavlovic 8 , Juliette D Godin 1 2 3 4 , Binnaz Yalcin 1 2 3 4 , Yann Hérault 1 2 3 4 , Pavel Dráber 5 , Jamel Chelly 1 2 3 4 9 10 , Maria-Victoria Hinckelmann 11

    Nature communications 13 May 2019 2019 ; : .

  • TUBG1 missense variants underlying cortical malformations disrupt neuronal locomotion and microtubule dynamics but not neurogenesis.

    Ivanova EL(1)(2)(3)(4), Gilet JG(1)(2)(3)(4), Sulimenko V(5), Duchon A(1)(2)(3)(4), Rudolf G(1)(2)(3)(4), Runge K(1)(2)(3)(4), Collins SC(1)(2)(3)(4)(6), Asselin L(1)(2)(3)(4), Broix L(1)(2)(3)(4), Drouot N(1)(2)(3)(4), Tilly P(1)(2)(3)(4), Nusbaum P(7), Vincent A(1)(2)(4), Magnant W(1)(2)(4), Skory V(1)(2)(3)(4), Birling MC(8), Pavlovic G(8), Godin JD(1)(2)(3)(4), Yalcin B(1)(2)(3)(4), Herault Y(1)(2)(3)(4), Draber P(5), Chelly J(1)(2)(3)(4)(9)(10), Hinckelmann MV(11)(12)(13)(14).

    Nat Commun May 13, 2019 ; 10:2129 .

  • Neutrophils recruited by leukotriene B4 induce features of plaque destabilization during endotoxemia.

    Mawhin MA(1)(2), Tilly P(2), Zirka G(1), Charles AL(3), Slimani F(2), Vonesch JL(4), Michel JB(1), Back M(5)(6), Norel X(1), Fabre JE(1).

    Cardiovasc Res Oct. 1, 2018 ; 114:1656-1666 .

  • p27Kip1 Modulates Axonal Transport by Regulating alpha-Tubulin Acetyltransferase 1 Stability.

    Morelli G(1), Even A(2), Gladwyn-Ng I(3), Le Bail R(3), Shilian M(2), Godin JD(4), Peyre E(3), Hassan BA(5), Besson A(6), Rigo JM(7), Weil M(2), Brone B(7), Nguyen L(8).

    Cell Rep May 22, 2018 ; 23:2429-2442 .

  • Ciliogenesis and cell cycle alterations contribute to KIF2A-related malformations of cortical development.

    Broix L(1)(2)(3)(4)(5), Asselin L(1)(2)(3)(4), Silva CG(6), Ivanova EL(1)(2)(3)(4), Tilly P(1)(2)(3)(4), Gilet JG(1)(2)(3)(4), Lebrun N(5), Jagline H(1)(2)(3)(4), Muraca G(5), Saillour Y(5), Drouot N(1)(2)(3)(4), Reilly ML(7)(8)(9), Francis F(10)(11)(12), Benmerah A(8)(9), Bahi-Buisson N(7)(8), Belvindrah R(10)(11)(12), Nguyen L(6), Godin JD(1)(2)(3)(4), Chelly J(1)(2)(3)(4)(13), Hinckelmann MV(1)(2)(3)(4).

    Hum Mol Genet Jan. 15, 2018 ; 27:224-238 .

  • WD40-repeat 47, a microtubule-associated protein, is essential for brain development and autophagy.

    Kannan M(1)(2)(3)(4)(5), Bayam E(1)(2)(3)(4), Wagner C(1)(2)(3)(4), Rinaldi B(6), Kretz PF(1)(2)(3)(4), Tilly P(1)(2)(3)(4), Roos M(7), McGillewie L(8), Bar S(6), Minocha S(5), Chevalier C(1)(2)(3)(4), Po C(9); Sanger Mouse Genetics Project, Chelly J(1)(2)(3)(4), Mandel JL(1)(2)(3)(4), Borgatti R(10), Piton A(1)(2)(3)(4), Kinnear C(8), Loos B(7), Adams DJ(11), Herault Y(1)(2)(3)(4), Collins SC(1)(2)(3)(4)(12), Friant S(6), Godin JD(1)(2)(3)(4), Yalcin B(13)(2)(3)(4).

    Proc Natl Acad Sci U S A Oct. 12, 2017 ; 114:E9308-E9317 .

  • Retinoic acid controls early neurogenesis in the developing mouse cerebral cortex.

    Haushalter C(1), Asselin L(1), Fraulob V(1), Dolle P(2), Rhinn M(3).

    Dev Biol Oct. 1, 2017 ; 430:129-141 .

  • Dopaminergic neurons differentiating from LRRK2 G2019S induced pluripotent stem cells show early neuritic branching defects.

    Borgs L(1), Peyre E(1), Alix P(1), Hanon K(1), Grobarczyk B(1), Godin JD(1), Purnelle A(1), Krusy N(1), Maquet P(1,)(2), Lefebvre P(1,)(3), Seutin V(1), Malgrange B(1), Nguyen L(1).

    Sci Rep Sept. 19, 2016 ; 6:33377 .

  • Mutations in the HECT domain of NEDD4L lead to AKT-mTOR pathway deregulation and cause periventricular nodular heterotopia.

    Broix L(1,)(2,)(3,)(4,)(5), Jagline H(1,)(2,)(3,)(4), L Ivanova E(1,)(2,)(3,)(4), Schmucker S(1,)(2,)(3,)(4), Drouot N(1,)(2,)(3,)(4), Clayton-Smith J(6), Pagnamenta AT(7), Metcalfe KA(6), Isidor B(8), Louvier UW(9), Poduri A(10), Taylor JC(7), Tilly P(1,)(2,)(3,)(4), Poirier K(5), Saillour Y(5), Lebrun N(5), Stemmelen T(1,)(2,)(3,)(4), Rudolf G(1,)(2,)(3,)(4), Muraca G(5), Saintpierre B(5), Elmorjani A(5); Deciphering Developmental Disorders study, Moïse M(11), Weirauch NB(12), Guerrini R(13), Boland A(14), Olaso R(14), Masson C(15), Tripathy R(16), Keays D(16), Beldjord C(17), Nguyen L(11), Godin J(1,)(2,)(3,)(4), Kini U(18), Nischké P(15), Deleuze JF(14), Bahi-Buisson N(19), Sumara I(1,)(2,)(3,)(4), Hinckelmann MV(1,)(2,)(3,)(4), Chelly J(1,)(2,)(3,)(4,)(20).

    Nat Genet nov 2016 2016 ; 48:1349-1358 .

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  • Role Of Kif21b In Brain Connectivity: Implication In Human Neurodevelopmental Disorder (H/F)