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HDAC1/2-Dependent P0 Expression Maintains Paranodal and Nodal Integrity Independently of Myelin Stability through Interactions with Neurofascins.
Brügger, Valérie; Engler, Stefanie; Pereira, Jorge A; Ruff, Sophie; Horn, Michael; Welzl, Hans; Münger, Emmanuelle; Vaquié, Adrien; Sidiropoulos, Páris N M; Egger, Boris; Yotovski, Peter; Filgueira, Luis; Somandin, Christian; Lühmann, Tessa C; D'Antonio, Maurizio; Yamaguchi, Teppei; Matthias, Patrick; Suter, Ueli; Jacob, Claire.
Afiliación
  • Brügger V; Department of Biology, University of Fribourg, Fribourg, Switzerland.
  • Engler S; Department of Biology, University of Fribourg, Fribourg, Switzerland; Institute of Molecular Health Sciences, Department of Biology, ETH Zurich, Zurich, Switzerland.
  • Pereira JA; Institute of Molecular Health Sciences, Department of Biology, ETH Zurich, Zurich, Switzerland.
  • Ruff S; Department of Biology, University of Fribourg, Fribourg, Switzerland.
  • Horn M; Institute of Molecular Health Sciences, Department of Biology, ETH Zurich, Zurich, Switzerland.
  • Welzl H; Department of Anatomy, University of Zurich, Zurich, Switzerland.
  • Münger E; Department of Biology, University of Fribourg, Fribourg, Switzerland.
  • Vaquié A; Department of Biology, University of Fribourg, Fribourg, Switzerland.
  • Sidiropoulos PN; Institute of Molecular Health Sciences, Department of Biology, ETH Zurich, Zurich, Switzerland.
  • Egger B; Department of Biology, University of Fribourg, Fribourg, Switzerland.
  • Yotovski P; Anatomy, Department of Medicine, University of Fribourg, Fribourg, Switzerland.
  • Filgueira L; Anatomy, Department of Medicine, University of Fribourg, Fribourg, Switzerland.
  • Somandin C; Institute of Molecular Health Sciences, Department of Biology, ETH Zurich, Zurich, Switzerland.
  • Lühmann TC; Department of Materials, Laboratory for Biologically Oriented Materials, ETH Zurich, Zurich, Switzerland.
  • D'Antonio M; Division of Genetics and Cell Biology, San Raffaele Scientific Institute, DIBIT, Milano, Italy.
  • Yamaguchi T; FMI for Biomedical Research, Novartis Research Foundation, Basel, Switzerland.
  • Matthias P; FMI for Biomedical Research, Novartis Research Foundation, Basel, Switzerland.
  • Suter U; Institute of Molecular Health Sciences, Department of Biology, ETH Zurich, Zurich, Switzerland.
  • Jacob C; Department of Biology, University of Fribourg, Fribourg, Switzerland.
PLoS Biol ; 13(9): e1002258, 2015.
Article en En | MEDLINE | ID: mdl-26406915
The pathogenesis of peripheral neuropathies in adults is linked to maintenance mechanisms that are not well understood. Here, we elucidate a novel critical maintenance mechanism for Schwann cell (SC)-axon interaction. Using mouse genetics, ablation of the transcriptional regulators histone deacetylases 1 and 2 (HDAC1/2) in adult SCs severely affected paranodal and nodal integrity and led to demyelination/remyelination. Expression levels of the HDAC1/2 target gene myelin protein zero (P0) were reduced by half, accompanied by altered localization and stability of neurofascin (NFasc)155, NFasc186, and loss of Caspr and septate-like junctions. We identify P0 as a novel binding partner of NFasc155 and NFasc186, both in vivo and by in vitro adhesion assay. Furthermore, we demonstrate that HDAC1/2-dependent P0 expression is crucial for the maintenance of paranodal/nodal integrity and axonal function through interaction of P0 with neurofascins. In addition, we show that the latter mechanism is impaired by some P0 mutations that lead to late onset Charcot-Marie-Tooth disease.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Enfermedad de Charcot-Marie-Tooth / Moléculas de Adhesión Celular / Proteína P0 de la Mielina / Vaina de Mielina / Factores de Crecimiento Nervioso Tipo de estudio: Prognostic_studies Límite: Animals / Humans Idioma: En Revista: PLoS Biol Asunto de la revista: BIOLOGIA Año: 2015 Tipo del documento: Article País de afiliación: Suiza Pais de publicación: Estados Unidos
Texto completo
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Enfermedad de Charcot-Marie-Tooth / Moléculas de Adhesión Celular / Proteína P0 de la Mielina / Vaina de Mielina / Factores de Crecimiento Nervioso Tipo de estudio: Prognostic_studies Límite: Animals / Humans Idioma: En Revista: PLoS Biol Asunto de la revista: BIOLOGIA Año: 2015 Tipo del documento: Article País de afiliación: Suiza Pais de publicación: Estados Unidos