Search Results - "DE BLEECKER, Jan L"

Effect of efgartigimod on muscle group subdomains in participants with generalized myasthenia gravis: post hoc analyses of the phase 3 pivotal ADAPT study by Bril, Vera, Howard, James F., Karam, Chafic, De Bleecker, Jan L., Murai, Hiroyuki, Utsugisawa, Kimiaki, Ulrichts, Peter, Brauer, Edward, Zhao, Sihui, Mantegazza, Renato, Vu, Tuan

“…Background and purpose Generalized myasthenia gravis (gMG) is a rare, chronic, neuromuscular autoimmune disease mediated by pathogenic immunoglobulin G (IgG)…”
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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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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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FAHN/SPG35: a narrow phenotypic spectrum across disease classifications by Rattay, Tim W, Lindig, Tobias, Baets, Jonathan, Smets, Katrien, Deconinck, Tine, Söhn, Anne S, Hörtnagel, Konstanze, Eckstein, Kathrin N, Wiethoff, Sarah, Reichbauer, Jennifer, Döbler-Neumann, Marion, Krägeloh-Mann, Ingeborg, Auer-Grumbach, Michaela, Plecko, Barbara, Münchau, Alexander, Wilken, Bernd, Janauschek, Marc, Giese, Anne-Katrin, De Bleecker, Jan L, Ortibus, Els, Debyser, Martine, Lopez de Munain, Adolfo, Pujol, Aurora, Bassi, Maria Teresa, D'Angelo, Maria Grazia, De Jonghe, Peter, Züchner, Stephan, Bauer, Peter, Schöls, Ludger, Schüle, Rebecca

“…The endoplasmic reticulum enzyme fatty acid 2-hydroxylase (FA2H) plays a major role in the formation of 2-hydroxy glycosphingolipids, main components of…”
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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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C-terminal frameshift variant of TDP-43 with pronounced aggregation-propensity causes rimmed vacuole myopathy but not ALS/FTD by Ervilha Pereira, Pedro, Schuermans, Nika, Meylemans, Antoon, LeBlanc, Pontus, Versluys, Lauren, Copley, Katie E., Rubien, Jack D., Altheimer, Christopher, Peetermans, Myra, Debackere, Elke, Vanakker, Olivier, Janssens, Sandra, Baets, Jonathan, Verhoeven, Kristof, Lammens, Martin, Symoens, Sofie, De Paepe, Boel, Barmada, Sami J., Shorter, James, De Bleecker, Jan L., Bogaert, Elke, Dermaut, Bart

“…Neuronal TDP-43-positive inclusions are neuropathological hallmark lesions in frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS). Pathogenic…”
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Description of a Novel Mechanism Possibly Explaining the Antiproliferative Properties of Glucocorticoids in Duchenne Muscular Dystrophy Fibroblasts Based on Glucocorticoid Receptor GR and NFAT5 by Herbelet, Sandrine, De Paepe, Boel, De Bleecker, Jan L

“…Glucocorticoids are drugs of choice in Duchenne muscular dystrophy (DMD), prolonging patients' ambulation. Their mode of action at the protein level is not…”
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Disease specificity of autoantibodies to cytosolic 5'-nucleotidase 1A in sporadic inclusion body myositis versus known autoimmune diseases by Herbert, Megan K, Stammen-Vogelzangs, Judith, Verbeek, Marcel M, Rietveld, Anke, Lundberg, Ingrid E, Chinoy, Hector, Lamb, Janine A, Cooper, Robert G, Roberts, Mark, Badrising, Umesh A, De Bleecker, Jan L, Machado, Pedro M, Hanna, Michael G, Plestilova, Lenka, Vencovsky, Jiri, van Engelen, Baziel G, Pruijn, Ger J M

“…The diagnosis of inclusion body myositis (IBM) can be challenging as it can be difficult to clinically distinguish from other forms of myositis, particularly…”
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Expanding the TDP-43 Proteinopathy Pathway From Neurons to Muscle: Physiological and Pathophysiological Functions by Versluys, Lauren, Ervilha Pereira, Pedro, Schuermans, Nika, De Paepe, Boel, De Bleecker, Jan L, Bogaert, Elke, Dermaut, Bart

“…TAR DNA-binding protein 43, mostly referred to as TDP-43 (encoded by the gene) is strongly linked to the pathogenesis of amyotrophic lateral sclerosis (ALS)…”
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Description of Osmolyte Pathways in Maturing Mdx Mice Reveals Altered Levels of Taurine and Sodium/Myo-Inositol Co-Transporters by Merckx, Caroline, Cosemans, Gwenny, Zschüntzsch, Jana, Raedt, Robrecht, Schmidt, Jens, De Paepe, Boel, De Bleecker, Jan L

“…Duchenne muscular dystrophy (DMD) is a genetic disorder characterized by progressive muscle degeneration. Osmotic stress participates to DMD pathology and…”
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Exploring the Therapeutic Potential of Ectoine in Duchenne Muscular Dystrophy: Comparison with Taurine, a Supplement with Known Beneficial Effects in the mdx Mouse by Merckx, Caroline, Zschüntzsch, Jana, Meyer, Stefanie, Raedt, Robrecht, Verschuere, Hanne, Schmidt, Jens, De Paepe, Boel, De Bleecker, Jan L.

“…Duchenne Muscular Dystrophy (DMD) is a debilitating muscle disorder that condemns patients to year-long dependency on glucocorticoids. Chronic glucocorticoid…”
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Loss of DPP6 in neurodegenerative dementia: a genetic player in the dysfunction of neuronal excitability by Cacace, Rita, Heeman, Bavo, Van Mossevelde, Sara, De Roeck, Arne, Hoogmartens, Julie, De Rijk, Peter, Gossye, Helena, De Vos, Kristof, De Coster, Wouter, Strazisar, Mojca, De Baets, Greet, Schymkowitz, Joost, Rousseau, Frederic, Geerts, Nathalie, De Pooter, Tim, Peeters, Karin, Sieben, Anne, Martin, Jean-Jacques, Engelborghs, Sebastiaan, Salmon, Eric, Santens, Patrick, Vandenberghe, Rik, Cras, Patrick, P. De Deyn, Peter, C. van Swieten, John, M. van Duijn, Cornelia, van der Zee, Julie, Sleegers, Kristel, Van Broeckhoven, Christine

“…Emerging evidence suggested a converging mechanism in neurodegenerative brain diseases (NBD) involving early neuronal network dysfunctions and alterations in…”
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Inducible Heat Shock Protein 70 Levels in Patients and the mdx Mouse Affirm Regulation during Skeletal Muscle Regeneration in Muscular Dystrophy by Cosemans, Gwenny, Merckx, Caroline, De Bleecker, Jan L., De Paepe, Boel

“…Background: Stress-inducible heat shock protein 70 (HSP70) is both a protective chaperone involved in protein homeostasis and an immune regulator. In both…”
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NEK1 genetic variability in a Belgian cohort of ALS and ALS-FTD patients by Nguyen, Hung Phuoc, Van Mossevelde, Sara, Dillen, Lubina, De Bleecker, Jan L., Moisse, Matthieu, Van Damme, Philip, Van Broeckhoven, Christine, van der Zee, Julie, Engelborghs, Sebastiaan, Crols, Roeland, De Deyn, Peter P., De Jonghe, Peter, Baets, Jonathan, Cras, Patrick, Mercelis, Rudy, Vandenberghe, Rik, Sieben, Anne, Santens, Patrick, Ivanoiu, Adrian, Deryck, Olivier, Vanopdenbosch, Ludo, Delbeck, Jean

“…We evaluated the genetic impact of the amyotrophic lateral sclerosis (ALS) risk gene never in mitosis gene a–related kinase 1 (NEK1) in a Belgian cohort of 278…”
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205th ENMC International Workshop: Pathology diagnosis of idiopathic inflammatory myopathies Part II 28–30 March 2014, Naarden, The Netherlands by De Bleecker, Jan L, De Paepe, Boel, Aronica, Eleonora, de Visser, Marianne, Amato, Anthony, Benveniste, Olivier, De Bleecker, Jan, de Boer, Onno, Dimachkie, Mazen, Gherardi, Romain, Goebel, Hans Hilmar, Hilton-Jones, David, Holton, Janice, Lundberg, Ingrid E, Mammen, Andrew, Mastaglia, Frank, Nishino, Ichizo, Rushing, Elisabeth, Daa Schroder, Henrik, Selcen, Duygu, Stenzel, Werner

“…Highlights • The heterogeneous group of idiopathic inflammatory myopathies (IM) needs international consensus on myopathologic classification. • Experts agreed…”
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Retrospective Study Shows That Serum Levels of Chemokine CXCL10 and Cytokine GDF15 Support a Diagnosis of Sporadic Inclusion Body Myositis and Immune-Mediated Necrotizing Myopathy by De Paepe, Boel, Bracke, Ken R, De Bleecker, Jan L

“…The implementation of novel blood-based biomarkers is desired to reduce the diagnostic delay and burden for myositis patients. In this retrospective study, the…”
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Role of cytokines and chemokines in idiopathic inflammatory myopathies by De Paepe, Boel, Creus, Kim K, De Bleecker, Jan L

“…PURPOSE OF REVIEWCytokines and chemokines are essential players in the initiation and progression of the idiopathic inflammatory myopathies (IIMs). This review…”
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Long-term safety, tolerability, and efficacy of efgartigimod (ADAPT+): interim results from a phase 3 open-label extension study in participants with generalized myasthenia gravis by Howard, Jr, James F, Bril, Vera, Vu, Tuan, Karam, Chafic, Peric, Stojan, De Bleecker, Jan L, Murai, Hiroyuki, Meisel, Andreas, Beydoun, Said R, Pasnoor, Mamatha, Guglietta, Antonio, Van Hoorick, Benjamin, Steeland, Sophie, T'joen, Caroline, Utsugisawa, Kimiaki, Verschuuren, Jan, Mantegazza, Renato

“…ADAPT+ assessed the long-term safety, tolerability, and efficacy of efgartigimod in adult participants with generalized myasthenia gravis (gMG). ADAPT+ was an…”
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Advance care planning in amyotrophic lateral sclerosis (ALS): study protocol for a qualitative longitudinal study with persons with ALS and their family carers by Vandenbogaerde, Isabel, Miranda, Rose, De Bleecker, Jan L, Carduff, Emma, van der Heide, Agnes, Van den Block, Lieve, Deliens, Luc, De Vleminck, Aline

“…IntroductionAmyotrophic lateral sclerosis (ALS) is an incurable motor neuron degenerative disease that has rapid progression and is associated with cognitive…”
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Seronegative patients form a distinctive subgroup of immune-mediated necrotizing myopathy by Lim, Johan, Rietveld, Anke, De Bleecker, Jan L, Badrising, Umesh A, Saris, Christiaan G.J, van der Kooi, Anneke J, de Visser, Marianne

“…OBJECTIVESTo investigate the characteristics of different clinico-serologic subgroups of immune-mediated necrotizing myopathy (IMNM). METHODSWe retrospectively…”
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