Mitochondrial dysfunction underlying sporadic inclusion body myositis is ameliorated by the mitochondrial homing drug MA-5. [PDF]
Sporadic inclusion body myositis (sIBM) is the most common idiopathic inflammatory myopathy, and several reports have suggested that mitochondrial abnormalities are involved in its etiology. We recruited 9 sIBM patients and found significant histological
Yoshitsugu Oikawa +23 more
doaj +2 more sources
Treatment of sporadic inclusion body myositis with bimagrumab. [PDF]
To study activin signaling and its blockade in sporadic inclusion body myositis (sIBM) through translational studies and a randomized controlled trial.We measured transforming growth factor β signaling by SMAD2/3 phosphorylation in muscle biopsies of 50 patients with neuromuscular disease (17 with sIBM).
Amato AA +12 more
europepmc +4 more sources
Sporadic inclusion body myositis: new insights and potential therapy. [PDF]
To describe new insights and developments in the pathogenesis, diagnosis and treatment of sporadic inclusion body myositis (IBM).Various hypothesis about the pathogenesis of IBM continue to be investigated, including autoimmune factors, mitochondrial dysfunction, protein dyshomeostasis, altered nucleic acid metabolism, myonuclear degeneration and the ...
Machado PM, Dimachkie MM, Barohn RJ.
europepmc +4 more sources
CYLD dysregulation in pathogenesis of sporadic inclusion body myositis. [PDF]
AbstractSporadic inclusion body myositis (sIBM) is the most commonly acquired myopathy in middle-aged and elderly people. The muscle histology is characterized by both inflammation and degeneration, including sarcoplasmic aggregation of TDP-43. Cylindromatosis (CYLD) is a deubiquitinating enzyme that targets Lys63-linked ubiquitin chains and negatively
Yamashita S +10 more
europepmc +4 more sources
Dysphagia in Patients with Sporadic Inclusion Body Myositis: Management Challenges [PDF]
Nika Mohannak,1 Gemma Pattison,2 Kathryn Hird,1 Merrilee Needham1,3,4 1School of Medicine, The University of Notre Dame, Fremantle, Western Australia, Australia; 2Department of Speech Pathology, Royal Perth Hospital, Perth, Western Australia, Australia ...
Mohannak N +3 more
doaj +2 more sources
Proteomic study of sporadic inclusion body myositis. [PDF]
Sporadic inclusion body myositis (s-IBM) is the most commonly occurring acquired inflammatory myopathy in elderly people (>45 years); however, pathogenic mechanisms are poorly understood and diagnostic tools are limited. In view of this, new therapeutic and diagnostic molecular markers for s-IBM need to be identified.In this study, the proteomes from ...
Li K, Pu C, Huang X, Liu J, Mao Y, Lu X.
europepmc +4 more sources
Proteomic Profiling Unravels a Key Role of Specific Macrophage Subtypes in Sporadic Inclusion Body Myositis [PDF]
Unbiased proteomic profiling was performed toward the identification of biological parameters relevant in sIBM, thus giving hints about the pathophysiological processes and the existence of new reliable markers. For that purpose, skeletal muscle biopsies
Andreas Roos +6 more
doaj +2 more sources
Targeting protein homeostasis in sporadic inclusion body myositis. [PDF]
Augmenting the heat shock response with arimoclomol ameliorates pathology in cellular and animal models of inclusion body myositis.
Ahmed M +26 more
europepmc +6 more sources
The immune system in sporadic inclusion body myositis patients is not compromised by blood-flow restricted exercise training [PDF]
Background Sporadic inclusion body myositis (sIBM) is clinically characterised by progressive proximal and distal muscle weakness and impaired physical function while skeletal muscle tissue displays abnormal cellular infiltration of T cells, macrophages,
Kasper Yde Jensen +10 more
doaj +2 more sources
Mitochondrial and inflammatory changes in sporadic inclusion body myositis. [PDF]
AimsSporadic inclusion body myositis (sIBM) is the most common late onset muscle disease causing progressive weakness. In light of the lack of effective treatment, we investigated potential causes underlying muscle wasting. We hypothesized that accumulation of mitochondrial respiratory deficiency in muscle fibres may lead to fibre atrophy and ...
Rygiel KA +5 more
europepmc +5 more sources

