Search Results - "Plomp, J. J."

The expanding field of IgG4-mediated neurological autoimmune disorders by Huijbers, M. G., Querol, L. A., Niks, E. H., Plomp, J. J., van der Maarel, S. M., Graus, F., Dalmau, J., Illa, I., Verschuuren, J. J.

“…At least 13 different disease entities affecting the central nervous system, peripheral nervous system and connective tissue of the skin or kidneys are…”
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Natural disease history of mouse models for limb girdle muscular dystrophy types 2D and 2F by Pasteuning-Vuhman, S, Putker, K, Tanganyika-de Winter, C L, Boertje-van der Meulen, J W, van Vliet, L, Overzier, M, Plomp, J J, Aartsma-Rus, A, van Putten, M

“…Limb-girdle muscular dystrophy types 2D and 2F (LGMD 2D and 2F) are autosomal recessive disorders caused by mutations in the alpha- and delta sarcoglycan…”
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Unveiling contextual realities by microscopically entangling a neutron by Shen, J., Kuhn, S. J., Dalgliesh, R. M., de Haan, V. O., Geerits, N., Irfan, A. A. M., Li, F., Lu, S., Parnell, S. R., Plomp, J., van Well, A. A., Washington, A., Baxter, D. V., Ortiz, G., Snow, W. M., Pynn, R.

“…The development of qualitatively new measurement capabilities is often a prerequisite for critical scientific and technological advances. Here we introduce an…”
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MuSK IgG4 autoantibodies cause myasthenia gravis by inhibiting binding between MuSK and Lrp4 by Huijbers, Maartje G., Zhang, Wei, Klooster, Rinse, Niks, Erik H., Friese, Matthew B., Straasheijm, Kirsten R., Thijssen, Peter E., Vrolijk, Hans, Plomp, Jaap J., Vogels, Pauline, Losen, Mario, Van der Maarel, Silvère M., Burden, Steven J., Verschuuren, Jan J.

“…Myasthenia gravis (MG) is a severely debilitating autoimmune disease that is due to a decrease in the efficiency of synaptic transmission at neuromuscular…”
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IgG4‐mediated autoimmune diseases: a niche of antibody‐mediated disorders by Huijbers, Maartje G., Plomp, Jaap J., der Maarel, Silvère M., Verschuuren, Jan J.

“…Immunoglobulin 4 (IgG4) is one of four human IgG subclasses and has several unique functional characteristics. It exhibits low affinity for complement and for…”
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Pathogenic immune mechanisms at the neuromuscular synapse: the role of specific antibody‐binding epitopes in myasthenia gravis by Huijbers, M. G., Lipka, A. F., Plomp, J. J., Niks, E. H., Maarel, S. M., Verschuuren, J. J.

“…Autoantibodies against three different postsynaptic antigens and one presynaptic antigen at the neuromuscular junction are known to cause myasthenic syndromes…”
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Natural disease history of the dy2J mouse model of laminin α2 (merosin)-deficient congenital muscular dystrophy by Pasteuning-Vuhman, S, Putker, K, Tanganyika-de Winter, C L, Boertje-van der Meulen, J W, van Vliet, L, Overzier, M, Plomp, J J, Aartsma-Rus, A, van Putten, M

“…Merosin deficient congenital muscular dystrophy 1A (MDC1A) is a very rare autosomal recessive disorder caused by mutations in the LAMA2 gene leading to severe…”
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Visualizing the heterogeneous breakdown of a fractal microstructure during compaction by neutron dark-field imaging by Harti, R. P., Valsecchi, J., Trtik, P., Mannes, D., Carminati, C., Strobl, M., Plomp, J., Duif, C. P., Grünzweig, C.

“…Structural properties of cohesive powders are dominated by their microstructural composition. Powders with a fractal microstructure show particularly…”
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Functional monovalency amplifies the pathogenicity of anti-MuSK IgG4 in myasthenia gravis by Vergoossen, Dana L. E., Plomp, Jaap J., Gstöttner, Christoph, Fillié-Grijpma, Yvonne E., Augustinus, Roy, Verpalen, Robyn, Wuhrer, Manfred, Parren, Paul W. H. I., Dominguez-Vega, Elena, van der Maarel, Silvère M., Verschuuren, Jan J., Huijbers, Maartje G.

“…Human immunoglobulin (Ig) G4 usually displays antiinflammatory activity, and observations of IgG4 autoantibodies causing severe autoimmune disorders are…”
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Synaptic Assembly of the Brain in the Absence of Neurotransmitter Secretion by Verhage, Matthijs, Maia, Ascanio S., Plomp, Jaap J., Brussaard, Arjen B., Heeroma, Joost H., Vermeer, Hendrika, Toonen, Ruud F., Hammer, Robert E., van den Berg, Timo K., Missler, Markus, Geuze, Hans J., Südhof, Thomas C.

“…Brain function requires precisely orchestrated connectivity between neurons. Establishment of these connections is believed to require signals secreted from…”
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A Cacna1a Knockin Migraine Mouse Model with Increased Susceptibility to Cortical Spreading Depression by van den Maagdenberg, Arn M.J.M, Pietrobon, Daniela, Pizzorusso, Tommaso, Kaja, Simon, Broos, Ludo A.M, Cesetti, Tiziana, van de Ven, Rob C.G, Tottene, Angelita, van der Kaa, Jos, Plomp, Jaap J, Frants, Rune R, Ferrari, Michel D

“…Migraine is a common, disabling, multifactorial, episodic neurovascular disorder of unknown etiology. Familial hemiplegic migraine type 1 (FHM-1) is a…”
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Muscle-specific kinase myasthenia gravis IgG4 autoantibodies cause severe neuromuscular junction dysfunction in mice by KLOOSTER, Rinse, PLOMP, Jaap J, MARTINEZ-MARTINEZ, Pilar, DE BAETS, Marc H, DER MAAREL, Silvère M. Van, VERSCHUUREN, Jan J, HUIJBERS, Maartje G, NIKS, Erik H, STRAASHEIJM, Kirsten R, DETMERS, Frank J, HERMANS, Pim W, SLEIJPEN, Kevin, VERRIPS, Aad, LOSEN, Mario

“…Myasthenia gravis is a paralytic disorder with autoantibodies against acetylcholine receptors at the neuromuscular junction. A proportion of patients instead…”
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Characterization of neuromuscular synapse function abnormalities in multiple Duchenne muscular dystrophy mouse models by van der Pijl, Elizabeth M., van Putten, Maaike, Niks, Erik H., Verschuuren, Jan J. G. M., Aartsma-Rus, Annemieke, Plomp, Jaap J.

“…Duchenne muscular dystrophy (DMD) is an X‐linked myopathy caused by dystrophin deficiency. Dystrophin is present intracellularly at the sarcolemma, connecting…”
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Pathophysiological actions of neuropathy-related anti-ganglioside antibodies at the neuromuscular junction by Plomp, Jaap J., Willison, Hugh J.

“…The outer leaflet of neuronal membranes is highly enriched in gangliosides. Therefore, specific neuronal roles have been attributed to this family of…”
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Electrophysiological analysis of neuromuscular synaptic function in myasthenia gravis patients and animal models by Plomp, Jaap J., Morsch, Marco, Phillips, William D., Verschuuren, Jan J.G.M.

“…Study of the electrophysiological function of the neuromuscular junction (NMJ) is instrumental in the understanding of the symptoms and pathophysiology of…”
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An automated hybrid bioelectronic system for autogenous restoration of sinus rhythm in atrial fibrillation by Nyns, Emile C A, Poelma, René H, Volkers, Linda, Plomp, Jaap J, Bart, Cindy I, Kip, Annemarie M, van Brakel, Thomas J, Zeppenfeld, Katja, Schalij, Martin J, Zhang, Guo Qi, de Vries, Antoine A F, Pijnappels, Daniël A

“…Because of suboptimal therapeutic strategies, restoration of sinus rhythm in symptomatic atrial fibrillation (AF) often requires in-hospital delivery of…”
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Particle size effects for carbon nanofiber supported platinum and ruthenium catalysts for the selective hydrogenation of cinnamaldehyde by Plomp, Arie J., Vuori, Heli, Krause, A. Outi I., de Jong, Krijn P., Bitter, Johannes H.

“…The selective hydrogenation of cinnamaldehyde was studied over carbon nanofibers (CNF) supported platinum and ruthenium catalysts. The catalysts differed…”
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Development and characterization of agonistic antibodies targeting the Ig-like 1 domain of MuSK by Lim, Jamie L., Augustinus, Roy, Plomp, Jaap J., Roya-Kouchaki, Kasra, Vergoossen, Dana L. E., Fillié-Grijpma, Yvonne, Struijk, Josephine, Thomas, Rachel, Salvatori, Daniela, Steyaert, Christophe, Blanchetot, Christophe, Vanhauwaert, Roeland, Silence, Karen, van der Maarel, Silvère M., Verschuuren, Jan J., Huijbers, Maartje G.

“…Muscle-specific kinase (MuSK) is crucial for acetylcholine receptor (AChR) clustering and thereby neuromuscular junction (NMJ) function. NMJ dysfunction is a…”
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Cross-sectional study into age-related pathology of mouse models for limb girdle muscular dystrophy types 2D and 2F by Verhaart, Ingrid E C, Putker, Kayleigh, van de Vijver, Davy, Tanganyika-de Winter, Christa L, Pasteuning-Vuhman, Svetlana, Plomp, Jaap J, Aartsma-Rus, Annemieke M, van Putten, Maaike

“…Limb girdle muscular dystrophy (LGMD) types 2D and 2F are caused by mutations in the genes encoding for α- and δ-sarcoglycan, respectively, leading to…”
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High cortical spreading depression susceptibility and migraine-associated symptoms in Cav2.1 S218L mice by van den Maagdenberg, Arn M. J. M., Pizzorusso, Tommaso, Kaja, Simon, Terpolilli, Nicole, Shapovalova, Maryna, Hoebeek, Freek E., Barrett, Curtis F., Gherardini, Lisa, van de Ven, Rob C. G., Todorov, Boyan, Broos, Ludo A. M., Tottene, Angelita, Gao, Zhenyu, Fodor, Mariann, De Zeeuw, Chris I., Frants, Rune R., Plesnila, Nikolaus, Plomp, Jaap J., Pietrobon, Daniela, Ferrari, Michel D.

“…Objective The CACNA1A gene encodes the pore‐forming subunit of neuronal CaV2.1 Ca2+ channels. In patients, the S218L CACNA1A mutation causes a dramatic…”
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