Histone acetyltransferase inhibition rescues differentiation of emerin-deficient myogenic progenitors.

ABSTRACT:

Introduction

Emery-Dreifuss muscular dystrophy (EDMD) is a disease characterized by skeletal muscle wasting, major tendon contractures, and cardiac conduction defects. Mutations in the gene encoding emerin cause EDMD1. Our previous studies suggested that emerin activation of histone deacetylase 3 (HDAC3) to reduce histone 4-lysine 5 (H4K5) acetylation (ac) is important for myogenic differentiation.

Methods

Pharmacological inhibitors (Nu9056, L002) of histone acetyltransferases targeting acetylated H4K5 were used to test whether increased acetylated H4K5 was responsible for the impaired differentiation seen in emerin-deficient myogenic progenitors.

Results

Nu9056 and L002 rescued impaired differentiation in emerin deficiency. SRT1720, which inhibits the nicotinamide ad

SUBMITTER: Bossone KA 

PROVIDER: S-EPMC7382944 | biostudies-literature | 2020 Jul

REPOSITORIES: biostudies-literature