Tolerability and efficacy study of P2X7 inhibition in experimental Charcot-Marie-Tooth type 1A (CMT1A) neuropathy
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- 作者：Giovanna Socialia ; Davide Visigallib ; Thomas Prukopc ; d ; Ilaria Cervellinic ; Elena Manninoa ; Consuelo Venturib ; Santina Bruzzonea ; santina.bruzzone@unige.it" class="auth_mail" title="E-mail the corresponding author ; Michael W. Seredac ; e ; Angelo Schenoneb
- 关键词：CMT1A ; Charcot-Marie-Tooth 1A ; SC ; Schwann cells ; PMP22 ; peripheral myelin protein 22 ; [Ca2+]i ; intracellular Ca2+ concentration ; BBG ; Brilliant Blue G ; DRG ; dorsal root ganglia ; MPZ ; myelin protein zero ; NCV ; nerve conduction velocity ; CMAP ; compound muscle action potential ; DML ; distal motor latency ; SNAP ; sensory nerve action potential
- 刊名：Neurobiology of Disease
- 出版年：2016
- 出版时间：November 2016
- 年：2016
- 卷：95
- 期：Complete
- 页码：145-157
- 全文大小：1357 K
文摘
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A438079 improved in vitro myelination of organotypic DRG cultures from CMT1A rats.
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In CMT1A rats, A438079 increased muscle strength and number of myelinated axons.
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A438079 reduced the SC differentiation defect in CMT1A rats.
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Inhibition of the P2X7 receptor is well tolerated in CMT1A rats.
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Antagonizing P2X7 may improve the clinical phenotype in the CMT1A neuropathy.