Search Results - "Kho, Ay Lin"

Obscurin Rho GEF domains are phosphorylated by MST-family kinases but do not exhibit nucleotide exchange factor activity towards Rho GTPases in vitro by Koch, Daniel, Kho, Ay Lin, Fukuzawa, Atsushi, Alexandrovich, Alexander, Vanaanen, Kutti J, Beavil, Andrew, Pfuhl, Mark, Rees, Martin, Gautel, Mathias

“…Obscurin is a giant muscle protein (>800 kDa) featuring multiple signalling domains, including an SH3-DH-PH domain triplet from the Trio-subfamily of guanosine…”
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Mechanoenzymatics of titin kinase by Puchner, Elias M, Alexandrovich, Alexander, Kho, Ay Lin, Hensen, Ulf, Schäfer, Lars V, Brandmeier, Birgit, Gräter, Frauke, Grubmüller, Helmut, Gaub, Hermann E, Gautel, Mathias

“…Biological responses to mechanical stress require strain-sensing molecules, whose mechanically induced conformational changes are relayed to signaling cascades…”
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The molecular basis for sarcomere organization in vertebrate skeletal muscle by Wang, Zhexin, Grange, Michael, Wagner, Thorsten, Kho, Ay Lin, Gautel, Mathias, Raunser, Stefan

“…Sarcomeres are force-generating and load-bearing devices of muscles. A precise molecular picture of how sarcomeres are built underpins understanding their role…”
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Structures from intact myofibrils reveal mechanism of thin filament regulation through nebulin by Wang, Zhexin, Grange, Michael, Pospich, Sabrina, Wagner, Thorsten, Kho, Ay Lin, Gautel, Mathias, Raunser, Stefan

“…In skeletal muscle, nebulin stabilizes and regulates the length of thin filaments, but the underlying mechanism remains nebulous. In this work, we used…”
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Glycated proteins stimulate reactive oxygen species production in cardiac myocytes : Involvement of Nox2 (gp91phox)-containing NADPH oxidase by MIN ZHANG, AY LIN KHO, ANILKUMAR, Narayana, CHIBBER, Rakesh, PAGANO, Patrick J, SHAH, Ajay M, CAVE, Alison C

“…Nonenzymatic glycation that results in the production of early-glycation Amadori-modified proteins and advanced-glycation end products may be important in the…”
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Making sense of missense variants in TTN-related congenital myopathies by Rees, Martin, Nikoopour, Roksana, Fukuzawa, Atsushi, Kho, Ay Lin, Fernandez-Garcia, Miguel A., Wraige, Elizabeth, Bodi, Istvan, Deshpande, Charu, Özdemir, Özkan, Daimagüler, Hülya-Sevcan, Pfuhl, Mark, Holt, Mark, Brandmeier, Birgit, Grover, Sarah, Fluss, Joël, Longman, Cheryl, Farrugia, Maria Elena, Matthews, Emma, Hanna, Michael, Muntoni, Francesco, Sarkozy, Anna, Phadke, Rahul, Quinlivan, Ros, Oates, Emily C., Schröder, Rolf, Thiel, Christian, Reimann, Jens, Voermans, Nicol, Erasmus, Corrie, Kamsteeg, Erik-Jan, Konersman, Chaminda, Grosmann, Carla, McKee, Shane, Tirupathi, Sandya, Moore, Steven A., Wilichowski, Ekkehard, Hobbiebrunken, Elke, Dekomien, Gabriele, Richard, Isabelle, Van den Bergh, Peter, Domínguez-González, Cristina, Cirak, Sebahattin, Ferreiro, Ana, Jungbluth, Heinz, Gautel, Mathias

“…Mutations in the sarcomeric protein titin, encoded by TTN , are emerging as a common cause of myopathies. The diagnosis of a TTN -related myopathy is, however,…”
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Molecular noise filtering in the β-adrenergic signaling network by phospholamban pentamers by Koch, Daniel, Alexandrovich, Alexander, Funk, Florian, Kho, Ay Lin, Schmitt, Joachim P., Gautel, Mathias

“…Phospholamban (PLN) is an important regulator of cardiac calcium handling due to its ability to inhibit the calcium ATPase SERCA. β-Adrenergic stimulation…”
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Formin follows function: a muscle-specific isoform of FHOD3 is regulated by CK2 phosphorylation and promotes myofibril maintenance by Iskratsch, Thomas, Lange, Stephan, Dwyer, Joseph, Kho, Ay Lin, Remedios, Cris dos, Ehler, Elisabeth

“…Members of the formin family are important for actin filament nucleation and elongation. We have identified a novel striated muscle-specific splice variant of…”
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The Cardiac Stress Response Factor Ms1 Can Bind to DNA and Has a Function in the Nucleus by Zaleska, Mariola, Fogl, Claudia, Kho, Ay Lin, Ababou, Abdessamad, Ehler, Elisabeth, Pfuhl, Mark

“…Ms1 (also known as STARS and ABRA) has been shown to act as an early stress response gene in processes as different as hypertrophy in skeletal and cardiac…”
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Structure of the native myosin filament in the relaxed cardiac sarcomere by Tamborrini, Davide, Wang, Zhexin, Wagner, Thorsten, Tacke, Sebastian, Stabrin, Markus, Grange, Michael, Kho, Ay Lin, Rees, Martin, Bennett, Pauline, Gautel, Mathias, Raunser, Stefan

“…The thick filament is a key component of sarcomeres, the basic units of striated muscle 1 . Alterations in thick filament proteins are associated with familial…”
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Missense mutations in the central domains of cardiac myosin binding protein-C and their potential contribution to hypertrophic cardiomyopathy by Pearce, Amy, Ponnam, Saraswathi, Holt, Mark R, Randall, Thomas, Beckingham, Rylan, Kho, Ay Lin, Kampourakis, Thomas, Ehler, Elisabeth

“…Myosin binding protein-C (MyBP-C) is a multidomain protein that regulates muscle contraction. Mutations in MYBPC3, the gene encoding for the cardiac variant…”
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Recessive mutations in EPG5 cause Vici syndrome, a multisystem disorder with defective autophagy by Cullup, Thomas, Kho, Ay Lin, Dionisi-Vici, Carlo, Brandmeier, Birgit, Smith, Frances, Urry, Zoe, Simpson, Michael A, Yau, Shu, Bertini, Enrico, McClelland, Verity, Al-Owain, Mohammed, Koelker, Stefan, Koerner, Christian, Hoffmann, Georg F, Wijburg, Frits A, Hoedt, Amber E ten, Rogers, R Curtis, Manchester, David, Miyata, Rie, Hayashi, Masaharu, Said, Elizabeth, Soler, Doriette, Kroisel, Peter M, Windpassinger, Christian, Filloux, Francis M, Al-Kaabi, Salwa, Hertecant, Jozef, Del Campo, Miguel, Buk, Stefan, Bodi, Istvan, Goebel, Hans-Hilmar, Sewry, Caroline A, Abbs, Stephen, Mohammed, Shehla, Josifova, Dragana, Gautel, Mathias, Jungbluth, Heinz

“…Heinz Jungbluth and colleagues report the identification of mutations in EPG5 that cause Vici syndrome, characterized by callosal agenesis, cataracts,…”
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Recessive TTN truncating mutations define novel forms of core myopathy with heart disease by Chauveau, Claire, Bonnemann, Carsten G, Julien, Cedric, Kho, Ay Lin, Marks, Harold, Talim, Beril, Maury, Philippe, Arne-Bes, Marie Christine, Uro-Coste, Emmanuelle, Alexandrovich, Alexander, Vihola, Anna, Schafer, Sebastian, Kaufmann, Beth, Medne, Livija, Hübner, Norbert, Foley, A Reghan, Santi, Mariarita, Udd, Bjarne, Topaloglu, Haluk, Moore, Steven A, Gotthardt, Michael, Samuels, Mark E, Gautel, Mathias, Ferreiro, Ana

“…Core myopathies (CM), the main non-dystrophic myopathies in childhood, remain genetically unexplained in many cases. Heart disease is not considered part of…”
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The sarcomeric cytoskeleton as a target for pharmacological intervention by Kho, Ay Lin, Perera, Sue, Alexandrovich, Alexander, Gautel, Mathias

“…Graphical abstract Highlights ► Actin and myosin filaments are paradigmatic examples of sarcomeric drug targets. ► Sarcomeric protein kinases control muscle…”
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Obscurin Rho GEF domains are phosphorylated by MST-family kinases but do not exhibit nucleotide exchange factor activity towards Rho GTPases in vitro by Daniel Koch, Ay Lin Kho, Atsushi Fukuzawa, Alexander Alexandrovich, Kutti J. Vanaanen, Andrew Beavil, Mark Pfuhl, Martin Rees, Mathias Gautel

“…Obscurin is a giant muscle protein (>800 kDa) featuring multiple signalling domains, including an SH3-DH-PH domain triplet from the Trio-subfamily of guanosine…”
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Mechanosignalling by Cytoskeletal Protein Kinases and their Disease Implications by Gautel, Mathias, Kho, Ay Lin, Alexandrovich, Alexander, Pippig, Diana, Gaub, Hermann

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Formin follows function: a muscle-specific isoform of FHOD3 is regulated by CK2 phosphorylation and promotes myofibril maintenance by Iskratsch, Thomas, Lange, Stephan, Dwyer, Joseph, Kho, Ay Lin, Remedios, Cris dos, Ehler, Elisabeth

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The Cardiac Stress Response Factor Ms1 Can Bind to DNA and Has a Function in the Nucleus: e0144614 by Zaleska, Mariola, Fogl, Claudia, Kho, Ay Lin, Ababou, Abdessamad, Ehler, Elisabeth, Pfuhl, Mark

“…Ms1 (also known as STARS and ABRA) has been shown to act as an early stress response gene in processes as different as hypertrophy in skeletal and cardiac…”
Get full text