Immunobiology of Inherited Muscular Dystrophies

Comprehensive Physiology
James G TidballMichelle Wehling-Henricks

Abstract

The immune response to acute muscle damage is important for normal repair. However, in chronic diseases such as many muscular dystrophies, the immune response can amplify pathology and play a major role in determining disease severity. Muscular dystrophies are inheritable diseases that vary tremendously in severity, but share the progressive loss of muscle mass and function that can be debilitating and lethal. Mutations in diverse genes cause muscular dystrophy, including genes that encode proteins that maintain membrane strength, participate in membrane repair, or are components of the extracellular matrix or the nuclear envelope. In this article, we explore the hypothesis that an important feature of many muscular dystrophies is an immune response adapted to acute, infrequent muscle damage that is misapplied in the context of chronic injury. We discuss the involvement of the immune system in the most common muscular dystrophy, Duchenne muscular dystrophy, and show that the immune system influences muscle death and fibrosis as disease progresses. We then present information on immune cell function in other muscular dystrophies and show that for many muscular dystrophies, release of cytosolic proteins into the extracellular spa...Continue Reading

Citations

Mar 20, 2019·Journal of Muscle Research and Cell Motility·Sandra MurphyKay Ohlendieck
Jul 31, 2019·Expert Review of Molecular Diagnostics·Paul DowlingKay Ohlendieck
Feb 19, 2020·Expert Review of Proteomics·Paul DowlingKay Ohlendieck
Apr 1, 2020·Disease Models & Mechanisms·Miranda D GroundsPeter G Arthur
Aug 23, 2020·International Journal of Molecular Sciences·Chiara PanicucciClaudio Bruno
Nov 19, 2019·Frontiers in Physiology·J Spencer HauckJill A Rafael-Fortney
Jan 27, 2021·The American Journal of Pathology·Zachary M HowardJill A Rafael-Fortney
Feb 20, 2021·Nature Reviews. Disease Primers·Dongsheng DuanAnnemieke Aartsma-Rus
Mar 13, 2021·European Journal of Translational Myology·Paul DowlingKay Ohlendieck
Apr 8, 2021·Cellular and Molecular Life Sciences : CMLS·Alicja Starosta, Patryk Konieczny
May 12, 2021·Neuropathology and Applied Neurobiology·Heike KölbelWerner Stenzel
Aug 28, 2021·Life·Michael OgundeleYetrib Hathout
Sep 24, 2021·Pflügers Archiv : European journal of physiology·Kay Ohlendieck, Dieter Swandulla

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