A novel histone deacetylase 6 inhibitor improves myelination of Schwann cells in a model of Charcot–Marie–Tooth disease type 1A

• DOI: 10.1111/bph.15231
• Publication Date: 2020-08-15T16:36:26Z
• Authors: Nina Ha; Young Il Choi; Namhee Jung; Ju Young Song; Dae Kwon Bae; Min Cheol Kim; Yong Jae Lee; Hyeseung Song; Geon Kwak; Soyeon Jeong; Saeyoung Park; Soo Hyun Nam; Sung‐Chul Jung; Byung‐Ok Choi
• Keywords: HDAC6; Schwann cell; Histone deacetylase inhibitor

Charcot-Marie-Tooth (CMT) disease is the most common hereditary peripheral neuropathy. CMT type 1A (CMT1A) accounts for approximately 50% of patients and linked to PMP22 gene duplication. Histone deacetylase-6 (HDAC6) has pleiotropic effects, such as regulating lipid homeostasis cellular stress. Although HDAC6 been regarded a promising drug target neurodegenerative diseases, its inhibition not yet tested in CMT1A. Here we have therapeutic potential CKD-504, clinical stage inhibitor, mouse model CMT1A EXPERIMENTAL APPROACH: The potency selectivity CKD-504 was evaluated, using HDAC enzyme panel assay western blots. evaluated behavioural testing electrophysiological assessments C22 protein expression aggregation were analysed mesenchymal stem cell-derived Schwann cells from sciatic nerves mice.