Nicolas CHARLET BERGUERAND

Publications

• 2014

  • Article in a journal

    N‐ WASP is required for Amphiphysin‐2/ BIN 1‐dependent nuclear positioning and triad organization in skeletal muscle and is involved in the pathophysiology of centronuclear myopathy

    • Sestina Falcone
    • William Roman
    • Karim Hnia
    • Vincent Gache
    • Nathalie Didier
    • Jeanne Lainé
    • Frederic Auradé
    • Isabelle Marty
    • Ichizo Nishino
    • Nicolas Charlet‐berguerand
    • Norma Beatriz Romero
    • Giovanna Marazzi
    • David Sassoon
    • Jocelyn Laporte
    • Edgar Gomes

    EMBO Molecular Medicine ; Volume: 6 ; Page: 1455-1475

    HAL DOI

  • Article in a journal

    FXTAS: Size does matter!

    • Chantal Sellier
    • Nicolas Charlet-Berguerand

    Cell Cycle ; Volume: 13 ; Page: 3319-3319

    HAL DOI

  • Article in a journal

    Tau exon 2 responsive elements deregulated in myotonic dystrophy type I are proximal to exon 2 and synergistically regulated by MBNL1 and MBNL2

    • C. Carpentier
    • D. Ghanem
    • F. J. Fernandez-Gomez
    • F. Jumeau
    • J. V. Philippe
    • F. Freyermuth
    • A. Labudeck
    • S. Eddarkaoui
    • C. M. Dhaenens
    • I. Holt
    • I. Behm-Ansmant
    • N. Marmier-Gourrier
    • C. Branlant
    • Nicolas Charlet-Berguerand
    • J. Marie
    • S. Schraen-Maschke
    • L. Buee
    • Nicolas Sergeant
    • Marie-Laure Caillet-Boudin

    Biochimica et Biophysica Acta - Molecular Basis of Disease ; Volume: 1842 ; Page: 654-664

    HAL DOI

  • Article in a journal

    Decreased DGCR8 Expression and miRNA Dysregulation in Individuals with 22q11.2 Deletion Syndrome

    • Chantal Sellier
    • Vicki Hwang
    • Ravi Dandekar
    • Blythe Durbin-Johnson
    • Nicolas Charlet-Berguerand
    • Bradley Ander
    • Frank Sharp
    • Kathleen Angkustsiri
    • Tony Simon
    • Flora Tassone

    PLoS ONE ; Volume: 9 ; Page: e103884

    HAL DOI

• 2013

  • Article in a journal

    The multiple molecular facets of fragile X-associated tremor/ataxia syndrome.

    • Chantal Sellier
    • Karen Usdin
    • Chiara Pastori
    • Veronica J Peschansky
    • Flora Tassone
    • Nicolas Charlet-Berguerand

    Journal of Neurodevelopmental Disorders ; Volume: 6 ; Page: 23

    HAL DOI

  • Article in a journal

    Modeling key pathological features of frontotemporal dementia with C9ORF72 repeat expansion in iPSC-derived human neurons

    • Sandra Almeida
    • Eduardo Gascon
    • Hélène Tran
    • Hsin Jung Chou
    • Tania Gendron
    • Steven Degroot
    • Andrew Tapper
    • Chantal Sellier
    • Nicolas Charlet-Berguerand
    • Anna Karydas
    • William Seeley
    • Adam Boxer
    • Leonard Petrucelli
    • Bruce Miller
    • Fen-Biao Gao

    Acta Neuropathologica ; Volume: 126 ; Page: 385-399

    HAL DOI

  • Article in a journal

    Sequestration of DROSHA and DGCR8 by Expanded CGG RNA Repeats Alters MicroRNA Processing in Fragile X-Associated Tremor/Ataxia Syndrome

    • Chantal Sellier
    • Fernande Freyermuth
    • Ricardos Tabet
    • Tuan Tran
    • Fang He
    • Frank Ruffenach
    • Violaine Alunni
    • Herve Moine
    • Christelle Thibault
    • Adeline Page
    • Flora Tassone
    • Rob Willemsen
    • Matthew d. Disney
    • Paul j. Hagerman
    • Peter k. Todd
    • Nicolas Charlet-Berguerand

    Cell Reports ; Volume: 3 ; Page: 869-880

    HAL DOI

  • Article in a journal

    CGG Repeat-Associated Translation Mediates Neurodegeneration in Fragile X Tremor Ataxia Syndrome

    • Peter K. Todd
    • Seok Yoon Oh
    • Amy Krans
    • Fang He
    • Chantal Sellier
    • Michelle Frazer
    • Abigail J. Renoux
    • Kai-Chun Chen
    • K. Matthew Scaglione
    • Venkatesha Basrur
    • Kojo Elenitoba-Johnson
    • Jean P. Vonsattel
    • Elan D. Louis
    • Michael A. Sutton
    • J. Paul Taylor
    • Ryan E. Mills
    • Nicolas Charlet-Berguerand
    • Henry L. Paulson

    Neuron ; Volume: 78 ; Page: 440--455

    HAL DOI

• 2012

  • Article in a journal

    A Small Molecule That Targets r(CGG) exp and Improves Defects in Fragile X-Associated Tremor Ataxia Syndrome

    • Matthew Disney
    • Biao Liu
    • Wang-Yong Yang
    • Chantal Sellier
    • Tuan Tran
    • Nicolas Charlet-Berguerand
    • Jessica Childs-Disney

    ACS Chemical Biology ; Volume: 7 ; Page: 1711-1718

    HAL DOI

• 2011

  • Article in a journal

    Misregulation of miR-1 processing is associated with heart defects in myotonic dystrophy

    • Frédérique Rau
    • Fernande Freyermuth
    • Charlotte Fugier
    • Jean-Philippe Villemin
    • Marie-Christine Fischer
    • Bernard Jost
    • Doulaye Dembele
    • Geneviève Gourdon
    • Annie Nicole
    • Denis Duboc
    • Karim Wahbi
    • John Day
    • Harutoshi Fujimura
    • Masanori Takahashi
    • Didier Auboeuf
    • Natacha Dreumont
    • Denis Furling
    • Nicolas Charlet-Berguerand

    Nature Structural and Molecular Biology ; Volume: 18 ; Page: 840-845

    HAL DOI