Anisotropic mechanosensitive pathways in the diaphragm and their implications in muscular dystrophies

J Muscle Res Cell Motil. 2017 Dec;38(5-6):437-446. doi: 10.1007/s10974-017-9483-7. Epub 2017 Oct 6.

Abstract

The diaphragm is the "respiratory pump;" the muscle that generates pressure to allow ventilation. Diaphragm muscles play a vital function and thus are subjected to continuous mechanical loading. One of its peculiarities is the ability to generate distinct mechanical and biochemical responses depending on the direction through which the mechanical forces applied to it. Contractile forces originated from its contractile components are transmitted to other structural components of its muscle fibers and the surrounding connective tissue. The anisotropic mechanical properties of the diaphragm are translated into biochemical signals that are directionally mechanosensitive by mechanisms that appear to be unique to this muscle. Here, we reviewed the current state of knowledge on the biochemical pathways regulated by mechanical signals emphasizing their anisotropic behavior in the normal diaphragm and analyzed how they are affected in muscular dystrophies.

Keywords: Mechanotransduction; Muscular dystrophy; Respiratory muscles.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, U.S. Gov't, Non-P.H.S.
  • Review

MeSH terms

  • Animals
  • Diaphragm* / metabolism
  • Diaphragm* / pathology
  • Diaphragm* / physiopathology
  • Humans
  • Muscle Contraction*
  • Muscle Strength*
  • Muscular Dystrophies* / metabolism
  • Muscular Dystrophies* / pathology
  • Muscular Dystrophies* / physiopathology