Next Article in Journal
High Glucose Represses the Anti-Proliferative and Pro-Apoptotic Effect of Metformin in Triple Negative Breast Cancer Cells
Previous Article in Journal
Toxicity Mechanism of Low Doses of NaGdF4:Yb3+,Er3+ Upconverting Nanoparticles in Activated Macrophage Cell Lines
Previous Article in Special Issue
Administration of Enalapril Started Late in Life Attenuates Hypertrophy and Oxidative Stress Burden, Increases Mitochondrial Mass, and Modulates Mitochondrial Quality Control Signaling in the Rat Heart
Article Menu

Export Article

Open AccessReview
Biomolecules 2019, 9(1), 15;

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)
Full-Text   |   PDF [2577 KB, uploaded 7 January 2019]   |  
  |   Review Reports


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

Figure 1

This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited (CC BY 4.0).

Share & Cite This Article

MDPI and ACS Style

De Paepe, B. Sporadic Inclusion Body Myositis: An Acquired Mitochondrial Disease with Extras. Biomolecules 2019, 9, 15.

Show more citation formats Show less citations formats

Note that from the first issue of 2016, MDPI journals use article numbers instead of page numbers. See further details here.

Related Articles

Article Metrics

Article Access Statistics



[Return to top]
Biomolecules EISSN 2218-273X Published by MDPI AG, Basel, Switzerland RSS E-Mail Table of Contents Alert
Back to Top