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Biomolecules 2019, 9(1), 15; https://doi.org/10.3390/biom9010015

Sporadic Inclusion Body Myositis: An Acquired Mitochondrial Disease with Extras

Neuromuscular Reference Centre, Ghent University Hospital, Corneel Heymanslaan 10, 9000 Ghent, Belgium
Received: 21 November 2018 / Revised: 19 December 2018 / Accepted: 19 December 2018 / Published: 7 January 2019
(This article belongs to the Special Issue Mitochondrial Diseases)
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Abstract

The sporadic form of inclusion body myositis (IBM) is the most common late-onset myopathy. Its complex pathogenesis includes degenerative, inflammatory and mitochondrial aspects. However, which of those mechanisms are cause and which effect, as well as their interrelations, remain partly obscured to this day. In this review the nature of the mitochondrial dysregulation in IBM muscle is explored and comparison is made with other muscle disorders. Mitochondrial alterations in IBM are evidenced by histological and serum biomarkers. Muscular mitochondrial dynamics is disturbed, with deregulated organelle fusion leading to subsequent morphological alterations and muscle displays abnormal mitophagy. The tissue increases mitochondrial content in an attempt to compensate dysfunction, yet mitochondrial DNA (mtDNA) alterations and mild mtDNA depletion are also present. Oxidative phosphorylation defects have repeatedly been shown, most notably a reduction in complex IV activities and levels of mitokines and regulatory RNAs are perturbed. Based on the cumulating evidence of mitochondrial abnormality as a disease contributor, it is therefore warranted to regard IBM as a mitochondrial disease, offering a feasible therapeutic target to be developed for this yet untreatable condition. View Full-Text
Keywords: mitochondrial dysfunction; myositis; sporadic inclusion body myositis mitochondrial dysfunction; myositis; sporadic inclusion body myositis
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De Paepe, B. Sporadic Inclusion Body Myositis: An Acquired Mitochondrial Disease with Extras. Biomolecules 2019, 9, 15.

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