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Inizio:  venerdì 27 aprile 2018 - Fine:  venerdì 27 aprile 2018
Titolo:  2018 IBP_CNR Seminars Series - April 27th, 2018
Dettagli:  

Institute of Protein Biochemistry, CNR Via P. Castellino, 111 - 80131 Napoli

2018 IBP Seminar Series

Friday, April 27th, 2018 at 2:30 p.m. - CNR Conference Room

Prof. Mario Bortolozzi (Venetian Institute of Molecular Medicine (VIMM) and University of Padua, Italy) will present:

Investigating peripheral neuropathies by a translational approach: from Physics to Medicine

Host: Dr. Alberto Luini - phone: 081-6132722; e-mail: a.luini@ibp.cnr.it

Abstract:
Mutations of connexin 32 (Cx32) protein cause the X-linked form of Charcot–Marie–Tooth disease (CMT1X), a demyelinating peripheral neuropathy for which there is no cure. A growing body of evidence indicates that ATP release through Cx32 hemichannels in Schwann cells could be critical for nerve myelination, but it is unknown if CMT1X mutations alter the cytosolic Ca2+-dependent gating mechanism that controls Cx32 hemichannel opening and ATP release. The current study uncovered a link between CMT1X and Cx32 hemichannel dysfunction, suggesting a candidate peptide for treating the disease caused by the R220X mutation. The study was carried out by a combination of in vitro fluorescence optical microscopy combined with patch clamp and in silico numerical simulations. Recently, we have complemented our biophysical approach by a cellular model based on induced pluripotent stem cells (iPSCs), derived from the same individual and differentiated into motor neurons and Schwann cells, which were subsequently co-cultured to mimic the peripheral nervous system.


Drs Maria Rosaria Coscia & Stefania Mariggiò
Seminar Coordinators Institute of Protein Biochemistry, CNR
Via P. Castellino, 111
80131 Naples Italy
Tel 0039 081 6132556/545

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