Search Results - "Paepe, Boel De"

Anti-Inflammatory and General Glucocorticoid Physiology in Skeletal Muscles Affected by Duchenne Muscular Dystrophy: Exploration of Steroid-Sparing Agents by Herbelet, Sandrine, Rodenbach, Arthur, Paepe, Boel De, De Bleecker, Jan L

“…In Duchenne muscular dystrophy (DMD), the activation of proinflammatory and metabolic cellular pathways in skeletal muscle cells is an inherent characteristic…”
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Scanning for Therapeutic Targets within the Cytokine Network of Idiopathic Inflammatory Myopathies by De Paepe, Boel, Zschüntzsch, Jana

“…The idiopathic inflammatory myopathies (IIM) constitute a heterogeneous group of chronic disorders that include dermatomyositis (DM), polymyositis (PM),…”
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Biallelic Mutations in TMEM126B Cause Severe Complex I Deficiency with a Variable Clinical Phenotype by Alston, Charlotte L., Compton, Alison G., Formosa, Luke E., Strecker, Valentina, Oláhová, Monika, Haack, Tobias B., Smet, Joél, Stouffs, Katrien, Diakumis, Peter, Ciara, Elżbieta, Cassiman, David, Romain, Nadine, Yarham, John W., He, Langping, De Paepe, Boel, Vanlander, Arnaud V., Seneca, Sara, Feichtinger, René G., Płoski, Rafal, Rokicki, Dariusz, Pronicka, Ewa, Haller, Ronald G., Van Hove, Johan L.K., Bahlo, Melanie, Mayr, Johannes A., Van Coster, Rudy, Prokisch, Holger, Wittig, Ilka, Ryan, Michael T., Thorburn, David R., Taylor, Robert W.

“…Complex I deficiency is the most common biochemical phenotype observed in individuals with mitochondrial disease. With 44 structural subunits and over 10…”
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Nanoscopic X‐ray imaging and quantification of the iron cellular architecture within single fibroblasts of Friedreich's ataxia patients by De Samber, Björn, Vanden Berghe, Tom, Meul, Eline, Bauters, Stephen, Seyrich, Martin, Smet, Joél, De Paepe, Boel, da Silva, Julio Cesar, Bohic, Sylvain, Cloetens, Peter, Van Coster, Rudy, Vandenabeele, Peter, Vincze, Laszlo

“…Friedreich's ataxia (FRDA) is a neurodegenerative disease characterized by an increase in intracytoplasmic iron concentration. Here the nanoscale iron…”
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Progressive Skeletal Muscle Atrophy in Muscular Dystrophies: A Role for Toll-like Receptor-Signaling in Disease Pathogenesis by De Paepe, Boel

“…Muscle atrophy is an active process controlled by specific transcriptional programs, in which muscle mass is lost by increased protein degradation and/or…”
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The Cytokine Growth Differentiation Factor-15 and Skeletal Muscle Health: Portrait of an Emerging Widely Applicable Disease Biomarker by De Paepe, Boel

“…Growth differentiation factor 15 (GDF-15) is a stress-induced transforming growth factor-β superfamily cytokine with versatile functions in human health…”
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Heat shock protein families 70 and 90 in Duchenne muscular dystrophy and inflammatory myopathy: Balancing muscle protection and destruction by De Paepe, Boel, Creus, Kim K, Weis, Joachim, De Bleecker, Jan L

“…Abstract Heat shock proteins are important factors in skeletal muscle physiology and stress response. We examined the effects of chronic inflammation on the…”
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How long noncoding RNAs enforce their will on mitochondrial activity: regulation of mitochondrial respiration, reactive oxygen species production, apoptosis, and metabolic reprogramming in cancer by De Paepe, Boel, Lefever, Steve, Mestdagh, Pieter

“…The cellular transcriptome contains a wide diversity of untranslated RNAs, of which the class of regulatory long noncoding RNAs (lncRNAs) has only recently…”
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Sporadic Inclusion Body Myositis: An Acquired Mitochondrial Disease with Extras by De Paepe, Boel

“…The sporadic form of inclusion body myositis (IBM) is the most common late-onset myopathy. Its complex pathogenesis includes degenerative, inflammatory and…”
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What Nutraceuticals Can Do for Duchenne Muscular Dystrophy: Lessons Learned from Amino Acid Supplementation in Mouse Models by De Paepe, Boel

“…Duchenne muscular dystrophy (DMD), the severest form of muscular dystrophy, is characterized by progressive muscle weakness with fatal outcomes most often…”
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The Role of Taurine in Skeletal Muscle Functioning and Its Potential as a Supportive Treatment for Duchenne Muscular Dystrophy by Merckx, Caroline, De Paepe, Boel

“…Taurine (2-aminoethanesulfonic acid) is required for ensuring proper muscle functioning. Knockout of the taurine transporter in mice results in low taurine…”
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How mitochondrial DNA-driven changes to chromosomal DNA methylation add a layer of complexity to mitochondrial disease by De Paepe, Boel

“…[...]human cells can display heteroplasmy of mtDNA, meaning different sequence variants may co-exist in the same cells. The clinical heterogeneity associated…”
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Incorporating circulating cytokines into the idiopathic inflammatory myopathy subclassification toolkit by De Paepe, Boel

“…Extensive diagnostic delays and deferred treatment impact the quality of life of patients suffering from an idiopathic inflammatory myopathy. In-depth…”
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Urine lactate concentration as a non-invasive screener for metabolic abnormalities: Findings in children with autism spectrum disorder and regression by Boterberg, Sofie, Vantroys, Elise, De Paepe, Boel, Van Coster, Rudy, Roeyers, Herbert

“…There is increasing evidence that diseases caused by dysfunctional mitochondria (MD) are associated with autism spectrum disorder (ASD). A comprehensive…”
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Abnormal NFAT5 Physiology in Duchenne Muscular Dystrophy Fibroblasts as a Putative Explanation for the Permanent Fibrosis Formation in Duchenne Muscular Dystrophy by Herbelet, Sandrine, De Paepe, Boel, De Bleecker, Jan L

“…Duchenne muscular dystrophy (DMD) is characterized by chronic inflammation and fibrotic tissue production by fibroblasts. The promyogenic factor nuclear factor…”
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Urine lactate concentration as a non-invasive screener for metabolic abnormalities: Findings in children with autism spectrum disorder and regression by Sofie Boterberg, Elise Vantroys, Boel De Paepe, Rudy Van Coster, Herbert Roeyers

“…There is increasing evidence that diseases caused by dysfunctional mitochondria (MD) are associated with autism spectrum disorder (ASD). A comprehensive…”
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POLG2 deficiency causes adult‐onset syndromic sensory neuropathy, ataxia and parkinsonism by Van Maldergem, Lionel, Besse, Arnaud, De Paepe, Boel, Blakely, Emma L., Appadurai, Vivek, Humble, Margaret M., Piard, Juliette, Craig, Kate, He, Langping, Hella, Pierre, Debray, François‐Guillaume, Martin, Jean‐Jacques, Gaussen, Marion, Laloux, Patrice, Stevanin, Giovanni, Van Coster, Rudy, Taylor, Robert W., Copeland, William C., Mormont, Eric, Bonnen, Penelope E.

“…Objective Mitochondrial dysfunction plays a key role in the pathophysiology of neurodegenerative disorders such as ataxia and Parkinson's disease. We describe…”
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Cytokines and Chemokines as Regulators of Skeletal Muscle Inflammation: Presenting the Case of Duchenne Muscular Dystrophy by De Paepe, Boel, De Bleecker, Jan L.

“…Duchenne muscular dystrophy is a severe inherited muscle disease that affects 1 in 3500 boys worldwide. Infiltration of skeletal muscle by inflammatory cells…”
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Hashimoto encephalopathy and antibodies against dimethylargininase-1: A rare cause of cognitive decline in a pediatric Down's syndrome patient by Helene, Verhelst, Patrick, Verloo, Boel, De Paepe, Gini, Beatrice, Bruno, Bonetti, Rudy, Van Coster

“…[...]it is not unusual to detect antibody markers such as antinuclear antibodies, rheumatoid factor and anti-gliadin antibodies in patients with HE. Ochi et…”
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Relevance of solute carrier family 5 transporter defects to inherited and acquired human disease by Cannizzaro, Miryam, Jarošová, Jana, De Paepe, Boel

“…The solute carrier (SLC) group of membrane transport proteins is crucial for cells via their control of import and export of vital molecules across the…”
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