Insights into muscle degeneration from heritable inclusion body myopathies

Frontiers in Aging Neuroscience
Sabine Krause

Abstract

Muscle mass and function are gradually lost in age-related, degenerative neuromuscular disorders, which also reflect the clinical hallmarks of sarcopenia. The consensus definition of sarcopenia includes a condition of age-related loss of muscle mass, quality, and strength. The most common acquired muscle disease affecting adults aged over 50 years is sporadic inclusion body myositis (sIBM). Besides inflammatory effects and immune-mediated muscle injury, degenerative myofiber changes are characteristic features of the disease. Although the earliest triggering events in sIBM remain elusive, a plethora of downstream mechanisms are implicated in the pathophysiology of muscle wasting. Although it remains controversial whether hereditary forms of inclusion body myopathy (IBM) may be considered as degenerative sIBM disease models, partial pathophysiological aspects can mimic the much more frequent sporadic condition, in particular the occurrence of inclusion bodies in skeletal muscle. Various clinical aspects in genetically determined skeletal muscle disorders reflect age-related alterations observed in sarcopenia. Several intriguing clues from monogenic defects in heritable IBMs contributing to the molecular basis of muscle loss will...Continue Reading

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Citations

Sep 1, 2015·Frontiers in Aging Neuroscience·Luciano MerliniEmanuele Marzetti
May 18, 2016·Free Radical Biology & Medicine·George G RodneyReem Abo-Zahrah
Feb 9, 2018·International Journal of Occupational Safety and Ergonomics : JOSE·Kristen A Rost, Alicia M Alvero
Apr 11, 2019·Journal of Cachexia, Sarcopenia and Muscle·Chun-Wei LiDong-Jing Li
Oct 31, 2018·Frontiers in Neuroscience·Shreedarshanee DeviRanjana Arya

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Methods Mentioned

BETA
ubiquitination
proteomic
biopsy
biopsies
protein folding

Software Mentioned

DAVID

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