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- Title
A dual role for Integrin α6β4 in modulating hereditary neuropathy with liability to pressure palsies.
- Authors
Poitelon, Yannick; Matafora, Vittoria; Silvestri, Nicholas; Zambroni, Desirée; McGarry, Claire; Serghany, Nora; Rush, Thomas; Vizzuso, Domenica; Court, Felipe A.; Bachi, Angela; Wrabetz, Lawrence; Feltri, Maria Laura
- Abstract
Abstract: Peripheral 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 <italic>PMP22</italic>, its function remains largely unknown. It was reported that <italic>PMP22</italic> interacts with the β4 subunit of the laminin receptor α6β4 integrin, suggesting that α6β4 integrin and laminins may contribute to the pathogenesis of CMT1A or HNPP. Here we asked if the lack of α6β4 integrin in Schwann cells influences myelin stability in the HNPP mouse model. Our data indicate that PMP22 and β4 integrin may not interact directly in myelinating Schwann cells, however, ablating β4 integrin delays the formation of tomacula, a characteristic feature of HNPP. In contrast, ablation of integrin β4 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.
- Subjects
PERIPHERAL neuropathy; INTEGRINS; MYELIN proteins; PERIPHERAL nervous system; LABORATORY mice
- Publication
Journal of Neurochemistry, 2018, Vol 145, Issue 3, p245
- ISSN
0022-3042
- Publication type
Article
- DOI
10.1111/jnc.14295