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dc.contributor.authorPoitelon, Yannick
dc.contributor.authorMatafora, Vittoria
dc.contributor.authorSilvestri, Nicholas
dc.contributor.authorZambroni, Desiree
dc.contributor.authorMcGarry, Claire
dc.contributor.authorSerghany, Nora
dc.contributor.authorRush, Thomas
dc.contributor.authorVizzuso, Domenica
dc.contributor.authorCourt, Felipe A. [Ctr Integrat Biol, Fac Sci, Universidad Mayor, Chile]
dc.contributor.authorBachi, Angela
dc.contributor.authorWrabetz, Lawrence
dc.contributor.authorFeltri, María Laura
dc.date.accessioned2021-02-03T18:13:44Z
dc.date.available2021-02-03T18:13:44Z
dc.date.issued2018-05
dc.identifier.citationPoitelon, Y., Matafora, V., Silvestri, N., Zambroni, D., McGarry, C., Serghany, N., Rush, T., Vizzuso, D., Court, F. A., Bachi, A., Wrabetz, L., & Feltri, M. L. (2018). A dual role for Integrin α6β4 in modulating hereditary neuropathy with liability to pressure palsies. Journal of neurochemistry, 145(3), 245–257. https://doi.org/10.1111/jnc.14295es
dc.identifier.issn0022-3042
dc.identifier.issneISSN: 1471-4159
dc.identifier.urihttp://repositorio.umayor.cl/xmlui/handle/sibum/7334
dc.identifier.urihttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC5924464/pdf/nihms933345.pdf
dc.identifier.urihttps://dx.doi.org/10.1111%2Fjnc.14295
dc.identifier.urihttp://europepmc.org/backend/ptpmcrender.fcgi?accid=PMC5924464&blobtype=pdf
dc.description.abstractPeripheral myelin protein 22 (PMP22) is a component of compact myelin in the peripheral nervous system. The amount of PMP22 in myelin is tightly regulated, and PMP22 over or under-expression cause Charcot-Marie-Tooth 1A (CMT1A) and Hereditary Neuropathy with Pressure Palsies (HNPP). Despite the importance of PMP22, its function remains largely unknown. It was reported that PMP22 interacts with the beta 4 subunit of the laminin receptor alpha 6 beta 4 integrin, suggesting that alpha 6 beta 4 integrin and laminins may contribute to the pathogenesis of CMT1A or HNPP. Here we asked if the lack of alpha 6 beta 4 integrin in Schwann cells influences myelin stability in the HNPP mouse model. Our data indicate that PMP22 and b4 integrin may not interact directly in myelinating Schwann cells, however, ablating beta 4 integrin delays the formation of tomacula, a characteristic feature of HNPP. In contrast, ablation of integrin beta 4 worsens nerve conduction velocities and non-compact myelin organization in HNPP animals. This study demonstrates that indirect interactions between an extracellular matrix receptor and a myelin protein influence the stability and function of myelinated fibers.es
dc.description.sponsorshipThis work was supported by Fondazione Telethon (GPP10007 to MLF and LW and GGP08021 to MLF) and the National Institutes of Health (NS045630 to MLF, NS096104 to LW). We thank Ueli Suter (Department of Biology, Institute of Molecular Health Sciences, ETH Zurich) for Pmp22-null mice, Filippo Giancotti (UT MD Anderson Cancer Center) for 4 integrin clones and 804G cell lines; Peter Brophy and Diane Sherman (Univ. of Edinburgh) for 4 integrin and periaxin antibodies, Shoichiro Tsukita (Kyoto University) for Claudin 19 antibody and Edward Hurley for semithin sections. Y.P, M.L.F designed research, analyzed and interpreted data; Y.P performed the majority of research; V.M and A.B performed mass spectrometry analysis; N.S performed electrophysiology analysis; D.Z. performed immunogold labeling; C.M., N.S, T.R. and D.V. provided technical assistance; Y.P and M.L.F wrote the manuscript. M.L.F and L.W. provided funding and scientific direction; L.W. critically reviewed the manuscript.es
dc.format.extent23 p., PDFes
dc.language.isoen_USes
dc.publisherWILEYes
dc.rightsAttribution-NonCommercial-NoDerivs 3.0 Chilees
dc.sourceJournal of neurochemistry, 145(3), 245–257
dc.subjectHNPPes
dc.subjectIntegrin beta 4es
dc.subjectMyelines
dc.subjectPeripheral nervees
dc.subjectPmp22es
dc.subjectSchwann cellses
dc.titleA dual role for Integrin alpha 6 beta 4 in modulating hereditary neuropathy with liability to pressure palsieses
dc.typeArtículo o Paperes
umayor.facultadHUMANIDADES
umayor.indizadorCOTes
umayor.politicas.sherpa/romeoLicence CC BY. Disponible en: https://v2.sherpa.ac.uk/id/publication/3653es
umayor.indexadoWeb of Sciencees
umayor.indexadoScopuses
umayor.indexadoWOS:000431005600005
umayor.indexadoPMID: 29315582
dc.identifier.doi10.1111/jnc.14295
umayor.indicadores.wos-(cuartil)Q1
umayor.indicadores.scopus-(scimago-sjr)SJR 1.83
umayor.indicadores.scopus-(scimago-sjr)SCIMAGO/ INDICE H: 221 H


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