Clarin‐2 is essential for hearing by maintaining stereocilia integrity and function

Autor: Aziz El-Amraoui, Stuart L. Johnson, Christine Petit, Michelle Simon, Prashanthini Jeyarajan, Lauren Chessum, Michael R. Bowl, Sherylanne Newton, Andrea Lelli, Helena Rr Wells, Frances M K Williams, Andrew Parker, Joanne Dorning, Didier Dulon, Susan Morse, Suhasini R. Gopal, Steve D.M. Brown, Sedigheh Delmaghani, Philomena Mburu, Christopher T. Esapa, Ronna Hertzano, Sara Wells, Debbie Williams, Carlos A. Aguilar, Kumar N. Alagramam, Pranav Patni, Thibault Peineau, Walter Marcotti, Laura F. Corns, Sally J. Dawson, Gemma F. Codner, Lucy A Dunbar

Publikováno v: EMBO Molecular Medicine
EMBO Molecular Medicine, Wiley Open Access, 2019, 11 (9), pp.e10288. ⟨10.15252/emmm.201910288⟩
EMBO Molecular Medicine, 2019, 11 (9), pp.e10288. ⟨10.15252/emmm.201910288⟩
EMBO Molecular Medicine, Vol 11, Iss 9, Pp n/a-n/a (2019)

International audience; Hearing relies on mechanically gated ion channels present in the actin-rich stereocilia bundles at the apical surface of cochlear hair cells. Our knowledge of the mechanisms underlying the formation and maintenance of the soun

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::edaae1d8c8cc8054398a9affa8ff41c6
https://hal-pasteur.archives-ouvertes.fr/pasteur-03261798/document

Structural Characterization of Whirlin Reveals an Unexpected and Dynamic Supramodule Conformation of Its PDZ Tandem

Autor: Christine Petit, Florent Delhommel, Benjamin Bardiaux, Julia Chamot-Rooke, Michael Nilges, Sébastien Brier, Florence Cordier, Amel Bahloul, Bertrand Raynal, Sylvie Nouaille, Nicolas Wolff, Baptiste Colcombet-Cazenave, Guillaume Bouvier

Publikováno v: Structure
Structure, Elsevier (Cell Press), 2017, 25 (11), pp.1645-1656.e5. ⟨10.1016/j.str.2017.08.013⟩
Structure, 2017, 25 (11), pp.1645-1656.e5. ⟨10.1016/j.str.2017.08.013⟩

International audience; Hearing relies on the transduction of sound-evoked vibrations into electric signals, occurring in the stereocilia bundle of hair cells. The bundle is organized in a staircase pattern formed by rows of packed stereocilia. This

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::cf6c0f0829c16de0cf89456ba92ad039
https://hal-pasteur.archives-ouvertes.fr/pasteur-02883914

Rubella Virus-Associated Cutaneous Granulomatous Disease : a Unique Complication in Immune-Deficient Patients, Not Limited to DNA Repair Disorders

Autor: Philippe Pérot, Hans D. Ochs, Bénédicte Neven, David Buchbinder, Jasjit Singh, Christine Bodemer, E.V. Deripapa, Louise E. Vaz, Despina Moshous, Fabian Hauck, Kathleen E. Sullivan, Christoph Klein, Anna Shcherbina, Michael H. Albert, Shahrzad Bakhtiar, Felipe Suarez, Anita Rack, Diane J. Nugent, Francisco A. Bonilla, Marc Eloit, Alfons Krol, Ludmila Perelygina, Mikko Seppänen

Publikováno v: Journal of Clinical Immunology
Journal of Clinical Immunology, 2019, 39 (1), pp.81-89. ⟨10.1007/s10875-018-0581-0⟩
J Clin Immunol
Journal of Clinical Immunology, Springer Verlag, 2019, 39 (1), pp.81-89. ⟨10.1007/s10875-018-0581-0⟩

International audience; The association of immunodeficiency-related vaccine-derived rubella virus (iVDRV) with cutaneous and visceral granulomatous disease has been reported in patients with primary immunodeficiency disorders (PIDs). The majority of

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::5ef272ce9bd72e6295c2cb501554a6f4
http://hdl.handle.net/10138/299978

The scalp hair collar and tuft signs: A retrospective multicenter study of 78 patients with a systematic review of the literature

Autor: C. Eschard, Jean-Philippe Lacour, Nausicaa Malissen, Juliette Mazereeuw-Hautier, Thomas Hubiche, Stéphanie Mallet, Emmanuel Mahé, Sébastien Barbarot, Valérie Rigau, Didier Bessis, Sylvie Fraitag, Ludovic Martin, Guillaume Captier, Nathalie Milla, C. Abasq, A. Phan, Marie-Christine Picot, Hassan El Fertit, Emmanuelle Bourrat, M. Marque, Christine Chiaverini, Olivia Boccara, Michèle Bigorre, Eve Puzenat, Nicolas Leboucq, Patrice Plantin, Pierre Vabres, F. Boralevi

Publikováno v: Journal of The American Academy of Dermatology
Journal of The American Academy of Dermatology, 2017, 76 (3), pp.478-487. ⟨10.1016/j.jaad.2016.08.046⟩
Journal of The American Academy of Dermatology, Elsevier, 2017, 76 (3), pp.478-487. ⟨10.1016/j.jaad.2016.08.046⟩

International audience; BACKGROUND:Hair collar sign (HCS) and hair tuft of the scalp (HTS) are cutaneous signs of an underlying neuroectodermal defect, but most available data are based on case reports.OBJECTIVE:We sought to define the clinical spect

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::c2b61debe2ae1ee9a99f094455c572a5
https://pubmed.ncbi.nlm.nih.gov/27742172

Cochlear outer hair cells undergo an apical circumference remodeling constrained by the hair bundle shape

Autor: Christine Petit, Dominique Weil, Michel Leibovici, Vincent Michel, Isabelle Foucher, Nadège Cayet, Raphaël Etournay, Jean-Pierre Hardelin, Jacques Boutet de Monvel, Léa Lepelletier

Publikováno v: Development (Cambridge, England)
Development (Cambridge, England), 2010, 137 (8), pp.1373-83. ⟨10.1242/dev.045138⟩
Development (Cambridge, England), Company of Biologists, 2010, 137 (8), pp.1373-83. ⟨10.1242/dev.045138⟩

International audience; Epithelial cells acquire diverse shapes relating to their different functions. This is particularly relevant for the cochlear outer hair cells (OHCs), whose apical and basolateral shapes accommodate the functioning of these ce

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::8846a56e4001addb44ed23b688f9dc3e
https://doi.org/10.1242/dev.045138