Inclusion body myositis
Ontology highlight
ABSTRACT:
ORGANISM(S): Homo sapiens
SUBMITTER: Pietro Fratta
PROVIDER: E-MTAB-2141 | biostudies-arrayexpress |
REPOSITORIES: biostudies-arrayexpress
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Project description:Immune cell infiltration in myositis were by examining microarray expression profiles in muscle biopsies from 31 myositis patients and 5 normal controls. Muscle samples from 36 subjects (5 normal controls, 5 NM, 8 DM, 8 PM and 10 IBM) were studied
2012-11-01 | E-GEOD-39454 | biostudies-arrayexpress
Project description:Expression profiling of human myositis muscle samples This study was designed to compare expression signatures among the various types of inflammatory myopathy, dermatomyositis (DM), inclusion body myositis (IBM), necrotizing myopathy (NM), nonspecific myopathy (NS), and polymyositis (PM) compared to normal (NL) muscle.
2019-08-30 | GSE128470 | GEO
Project description:Idiopathic inflammatory myopathies (polymyositis and dermatomyositis) are heterogeneous group of muscle disorders of unknown etiology.The pathogenic pathways responsible for muscle fiber damage and dysfunction in myositis are not currently well defined. Identification of such pathways may help to design novel therapeutic interventions and also help to develop diagnostic tests. Keywords: Development or differentiation design
2006-07-21 | GSE5370 | GEO
Project description:Microarray data from muscle biopsy specimens from subjects with inclusion body myositis, polymyositis, and normals Keywords: Research study
2005-10-01 | GSE3112 | GEO
Project description:MHC-I overexpression in muscle biopsies is a hallmark of inflammatory myopathies.However the mechanisms of MHC-I overexpression in each disease is not well understood. Microarray analysis from MHC-I-microdissected myofibers showed a differential expression signature in each inflammatory myopathy. Innate immunity and IFN-I pathways are upregulated vs healthy controls, specifically in dermatomyositis (DM). RNA from MHC-I-positive myofibers were obtained from muscle biopsies of 5 patients with dermatomyositis, 5 with polymyositis, 4 with inclusion body myositis and normal looking fibers from healthy controls.
2013-12-01 | E-GEOD-48280 | biostudies-arrayexpress
Project description:To identify changes in skeletal muscle microRNA expression after exercise and associate the identified microRNAs with mRNA and protein expression to disease-specific pathways in polymyositis and dermatomyositis
2017-10-31 | GSE95772 | GEO
Project description:To identify changes in skeletal muscle microRNA expression after exercise and associate the identified microRNAs with mRNA and protein expression to disease-specific pathways in polymyositis and dermatomyositis
2017-10-31 | GSE95735 | GEO
Project description:Microarray data from muscle biopsy specimens from subjects with inclusion body myositis, polymyositis, and normals Experiment Overall Design: Microarray experiments
2008-06-12 | E-GEOD-3112 | biostudies-arrayexpress
Project description:Septic patients treated in the intensive care unit (ICU) often develop multiple organ failure including persistent skeletal muscle dysfunction which results in the patientâs protracted recovery process. We have demonstrated that muscle mitochondrial enzyme activities are impaired in septic ICU patients resulting in decreased cellular energy which will interfere with muscle function and metabolism. Here we use detailed phenotyping and genomics to elucidate mechanisms leading to these impairments. Methodology/Principle Findings Utilising biopsy material from seventeen patients and ten age-matched controls we demonstrate that neither mitochondrial in vivo protein synthesis nor expression of mitochondrial genes are compromised. Indeed, there was partial activation of the mitochondrial biogenesis pathway involving NRF2?/GABP and its target genes TFAM, TFB1M and TFB2M yet clearly this failed to maintain mitochondrial function. We therefore utilised transcript profiling and pathway analysis of ICU patient skeletal muscle to generate insight into the molecular defects driving loss of muscle function and metabolic homeostasis. Gene ontology analysis of Affymetrix analysis demonstrated substantial loss of muscle specific genes, a global oxidative stress response related to most probably cytokine signalling, altered insulin related signalling and a substantial overlap between patients and muscle wasting/inflammatory animal models. MicroRNA 21 processing appeared defective suggesting that post-transcriptional protein synthesis regulation is altered by disruption of tissue microRNA expression. Finally, we were able to demonstrate that the phenotype of skeletal muscle in ICU patients is not merely one of inactivity, it appears to be an actively remodelling tissue, influenced by several mediators, all of which may be open to manipulation with the aim to improve clinical outcome. Conclusions/Significance This first combined protein and transcriptome based analysis of human skeletal muscle obtained from septic patients demonstrated that losses of mitochondria and muscle mass are accompanied by sustained protein synthesis (anabolic process) while dysregulation of transcription programmes appears to fail to compensate for increased damage and proteolysis. Our analysis identified both validated and novel clinically tractable targets to manipulate these failing processes and pursuit of these could lead to new potential treatments. Experiment Overall Design: 13 septic samples, 8 controls
2008-11-22 | E-GEOD-13205 | biostudies-arrayexpress
Project description:Neuromuscular disorders (NMDs) are clinically and genetically heterogenous and manifest as dystrophic, inflammatory and myopathic pathologies, among others. Our previous study on the cardiotoxin (CTX) mouse model of myodegeneration and inflammation linked muscle pathology with mitochondrial damage and oxidative stress. In this study, we investigated whether human NMDs display mitochondrial changes. Muscle biopsies from NMD patients, represented by dystrophic pathology [dysferlinopathy (dysfy); n=43], inflammatory pathology [inflammatory myopathy (IM); n=24] and myopathic pathology [distal myopathy with rimmer vacuoles (DMRV); n=31] were analysed. Mitochondrial damage was revealed in all NMDs by Enzyme histochemistry (SDH and SDH-COX deficiency), electron microscopy (vacuolation and abnormal ultrastructures) and biochemical assays (significantly increased ADP/ATP ratio). Proteomic analysis of muscle mitochondria from all three NMDs by Isobaric tag for relative and absolute quantitation (iTRAQ) labelling and liquid chromatography-tandem mass spectrometry (LC-MS/MS) analysis demonstrated down-regulation of electron transport chain (ETC) complex subunits, assembly factors and Krebs cycle enzymes. Most of the differentially expressed proteins were common among the three pathologies. Assay of ETC and Krebs cycle enzyme activities validated the MS data. Mitochondrial proteins from muscle pathologies also displayed higher trp oxidation and the same was corroborated in the CTX model. Molecular modelling predicted trp oxidation to alter the local structure of mitochondrial proteins. Our data highlight mitochondrial alterations in muscle pathologies, represented by structural changes, altered mitochondrial proteome and protein oxidation status, thereby establishing the importance of mitochondrial damage in human NMDs.
2016-04-04 | PXD002760 | Pride