Muscle development, regeneration and laminopathies: how lamins or lamina-associated proteins can contribute to muscle development, regeneration and disease

Abstract

The aim of this review article is to evaluate the current knowledge on associations between muscle formation and regeneration and components of the nuclear lamina. Lamins and their partners have become particularly intriguing objects of scientific interest since it has been observed that mutations in genes coding for these proteins lead to a wide range of diseases called laminopathies. For over the last 10 years, various laboratories worldwide have tried to explain the pathogenesis of these rare disorders. Analyses of the distinct aspects of laminopathies resulted in formulation of different hypotheses regarding the mechanisms of the development of these diseases. In the light of recent discoveries, A-type lamins—the main building blocks of the nuclear lamina—together with other key elements, such as emerin, LAP2α and nesprins, seem to be of great importance in the modulation of various signaling pathways responsible for cellular differentiation and proliferation.

Role of RNA Binding Proteins with prion-like domains in muscle and neuromuscular diseases

“…Furthermore, MATR3 binds to and regulates long non-coding RNA in muscles [134]. Last, MATR3 binds directly to Lamin A, a protein required for muscle differentiation [133,137,138]. Interestingly, mutations in LMNA gene encoding lamin A/C lead to skeletal and cardiac myopathy [139] and disrupt lamin A/ MATR3 interaction [133].…”

How Do Skeletal Muscles Die? An Overview

“…Therefore, the number of skeletal muscle cells is fixed after birth and when the cell dies, it will not be replaced. However, individual muscle cells can grow by increasing their diameter and the number of muscle fibers they contain (Dubinska-Magiera et al 2013).…”

Dysfunctional polycomb transcriptional repression contributes to lamin A/C–dependent muscular dystrophy

“…In particular, the study of lamin A/C is gaining an increasing interest for 3 reasons: (a) lamin A/C plays an undisputed role in several cellular processes from mechanotransduction to cell differentiation; (b) lamin A/C has a peculiar intranuclear distribution, being present in the nucleoplasm as well as in the nuclear periphery (5); (c) lamin A/C interacts with several epigenetic factors, exerting a functional control over transcriptional regulation (3,6). One of the most studied lamin A/Cdependent cellular process is myogenesis because mutations in the LMNA gene lead to muscular dystrophies, as in the case of Emery Dreifuss muscular dystrophy (EDMD) (7). However, epigenetic mechanisms involved in lamin-dependent dystrophy are still largely unknown.…”