Sporadic Inclusion Body Myositis: An Acquired Mitochondrial Disease with Extras
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...
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doaj-32003229b2674aeca8966791053d32322020-11-25T00:16:51ZengMDPI AGBiomolecules2218-273X2019-01-01911510.3390/biom9010015biom9010015Sporadic Inclusion Body Myositis: An Acquired Mitochondrial Disease with ExtrasBoel De Paepe0Neuromuscular Reference Centre, Ghent University Hospital, Corneel Heymanslaan 10, 9000 Ghent, BelgiumThe 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.http://www.mdpi.com/2218-273X/9/1/15mitochondrial dysfunctionmyositissporadic inclusion body myositis |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Boel De Paepe |
spellingShingle |
Boel De Paepe Sporadic Inclusion Body Myositis: An Acquired Mitochondrial Disease with Extras Biomolecules mitochondrial dysfunction myositis sporadic inclusion body myositis |
author_facet |
Boel De Paepe |
author_sort |
Boel De Paepe |
title |
Sporadic Inclusion Body Myositis: An Acquired Mitochondrial Disease with Extras |
title_short |
Sporadic Inclusion Body Myositis: An Acquired Mitochondrial Disease with Extras |
title_full |
Sporadic Inclusion Body Myositis: An Acquired Mitochondrial Disease with Extras |
title_fullStr |
Sporadic Inclusion Body Myositis: An Acquired Mitochondrial Disease with Extras |
title_full_unstemmed |
Sporadic Inclusion Body Myositis: An Acquired Mitochondrial Disease with Extras |
title_sort |
sporadic inclusion body myositis: an acquired mitochondrial disease with extras |
publisher |
MDPI AG |
series |
Biomolecules |
issn |
2218-273X |
publishDate |
2019-01-01 |
description |
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. |
topic |
mitochondrial dysfunction myositis sporadic inclusion body myositis |
url |
http://www.mdpi.com/2218-273X/9/1/15 |
work_keys_str_mv |
AT boeldepaepe sporadicinclusionbodymyositisanacquiredmitochondrialdiseasewithextras |
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1725382130980093952 |