dc.creatorPoitelon, Yannick
dc.creatorMatafora, Vittoria
dc.creatorSilvestri, Nicholas
dc.creatorZambroni, Desiree
dc.creatorMcGarry, Claire
dc.creatorSerghany, Nora
dc.creatorRush, Thomas
dc.creatorVizzuso, Domenica
dc.creatorCourt, Felipe A. [Ctr Integrat Biol, Fac Sci, Universidad Mayor, Chile]
dc.creatorBachi, Angela
dc.creatorWrabetz, Lawrence
dc.creatorFeltri, María Laura
dc.date.accessioned2021-02-03T18:13:44Z
dc.date.accessioned2022-10-18T18:42:54Z
dc.date.available2021-02-03T18:13:44Z
dc.date.available2022-10-18T18:42:54Z
dc.date.created2021-02-03T18:13:44Z
dc.date.issued2018-05
dc.identifierPoitelon, 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.14295
dc.identifier0022-3042
dc.identifiereISSN: 1471-4159
dc.identifierhttp://repositorio.umayor.cl/xmlui/handle/sibum/7334
dc.identifierhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC5924464/pdf/nihms933345.pdf
dc.identifierhttps://dx.doi.org/10.1111%2Fjnc.14295
dc.identifierhttp://europepmc.org/backend/ptpmcrender.fcgi?accid=PMC5924464&blobtype=pdf
dc.identifier10.1111/jnc.14295
dc.identifier.urihttps://repositorioslatinoamericanos.uchile.cl/handle/2250/4455118
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.
dc.languageen_US
dc.publisherWILEY
dc.rightsAttribution-NonCommercial-NoDerivs 3.0 Chile
dc.sourceJournal of neurochemistry, 145(3), 245–257
dc.subjectHNPP
dc.subjectIntegrin beta 4
dc.subjectMyelin
dc.subjectPeripheral nerve
dc.subjectPmp22
dc.subjectSchwann cells
dc.titleA dual role for Integrin alpha 6 beta 4 in modulating hereditary neuropathy with liability to pressure palsies
dc.typeArtículos de revistas


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