Search Results - "Fürst, Dieter O."

Cellular Mechanotransduction Relies on Tension-Induced and Chaperone-Assisted Autophagy by Ulbricht, Anna, Eppler, Felix J., Tapia, Victor E., van der Ven, Peter F.M., Hampe, Nico, Hersch, Nils, Vakeel, Padmanabhan, Stadel, Daniela, Haas, Albert, Saftig, Paul, Behrends, Christian, Fürst, Dieter O., Volkmer, Rudolf, Hoffmann, Bernd, Kolanus, Waldemar, Höhfeld, Jörg

“…Mechanical tension is an ever-present physiological stimulus essential for the development and homeostasis of locomotory, cardiovascular, respiratory, and…”
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Chaperone-Assisted Selective Autophagy Is Essential for Muscle Maintenance by Arndt, Verena, Dick, Nikolaus, Tawo, Riga, Dreiseidler, Michael, Wenzel, Daniela, Hesse, Michael, Fürst, Dieter O., Saftig, Paul, Saint, Robert, Fleischmann, Bernd K., Hoch, Michael, Höhfeld, Jörg

“…How are biological structures maintained in a cellular environment that constantly threatens protein integrity? Here we elucidate proteostasis mechanisms…”
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Angiotensin‐II type 1 receptor‐mediated Janus kinase 2 activation induces liver fibrosis by Granzow, Michaela, Schierwagen, Robert, Klein, Sabine, Kowallick, Benita, Huss, Sebastian, Linhart, Markus, Mazar, Irela G. Reza, Görtzen, Jan, Vogt, Annabelle, Schildberg, Frank A., Gonzalez‐Carmona, Maria A., Wojtalla, Alexandra, Krämer, Benjamin, Nattermann, Jacob, Siegmund, Sören V., Werner, Nikos, Fürst, Dieter O., Laleman, Wim, Knolle, Percy, Shah, Vijay H., Sauerbruch, Tilman, Trebicka, Jonel

“…Activation of the renin angiotensin system resulting in stimulation of angiotensin‐II (AngII) type I receptor (AT1R) is an important factor in the development…”
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Force-induced dephosphorylation activates the cochaperone BAG3 to coordinate protein homeostasis and membrane traffic by Ottensmeyer, Judith, Esch, Alessandra, Baeta, Henrique, Sieger, Sandro, Gupta, Yamini, Rathmann, Maximilian F, Jeschke, Andreas, Jacko, Daniel, Schaaf, Kirill, Schiffer, Thorsten, Rahimi, Bahareh, Lövenich, Lukas, Sisto, Angela, van der Ven, Peter F M, Fürst, Dieter O, Haas, Albert, Bloch, Wilhelm, Gehlert, Sebastian, Hoffmann, Bernd, Timmerman, Vincent, Huesgen, Pitter F, Höhfeld, Jörg

“…Proteome maintenance in contracting skeletal and cardiac muscles depends on the chaperone-regulating protein BAG3. Reduced BAG3 activity leads to muscle…”
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Filamin C-related myopathies: pathology and mechanisms by Fürst, Dieter O., Goldfarb, Lev G., Kley, Rudolf A., Vorgerd, Matthias, Olivé, Montse, van der Ven, Peter F. M.

“…The term filaminopathy was introduced after a truncating mutation in the dimerization domain of filamin C (FLNc) was shown to be responsible for a devastating…”
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The cochaperone BAG3 coordinates protein synthesis and autophagy under mechanical strain through spatial regulation of mTORC1 by Kathage, Barbara, Gehlert, Sebastian, Ulbricht, Anna, Lüdecke, Laura, Tapia, Victor E, Orfanos, Zacharias, Wenzel, Daniela, Bloch, Wilhelm, Volkmer, Rudolf, Fleischmann, Bernd K, Fürst, Dieter O, Höhfeld, Jörg

“…The cochaperone BAG3 is a central protein homeostasis factor in mechanically strained mammalian cells. It mediates the degradation of unfolded and damaged…”
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Myofibrillar Z-discs Are a Protein Phosphorylation Hot Spot with Protein Kinase C (PKCα) Modulating Protein Dynamics by Reimann, Lena, Wiese, Heike, Leber, Yvonne, Schwäble, Anja N., Fricke, Anna L., Rohland, Anne, Knapp, Bettina, Peikert, Christian D., Drepper, Friedel, van der Ven, Peter F.M., Radziwill, Gerald, Fürst, Dieter O., Warscheid, Bettina

“…The Z-disc is a protein-rich structure critically important for the development and integrity of myofibrils, which are the contractile organelles of…”
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Rho-kinase inhibitor coupled to peptide-modified albumin carrier reduces portal pressure and increases renal perfusion in cirrhotic rats by Klein, Sabine, Frohn, Franziska, Magdaleno, Fernando, Reker-Smit, Catharina, Schierwagen, Robert, Schierwagen, Irela, Uschner, Frank Erhard, van Dijk, Fransien, Fürst, Dieter O., Djudjaj, Sonja, Boor, Peter, Poelstra, Klaas, Beljaars, Leonie, Trebicka, Jonel

“…Rho-kinase (ROCK) activation in hepatic stellate cells (HSC) is a key mechanism promoting liver fibrosis and portal hypertension (PTH). Specific delivery of…”
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Atorvastatin inhibits proliferation and apoptosis, but induces senescence in hepatic myofibroblasts and thereby attenuates hepatic fibrosis in rats by Klein, Sabine, Klösel, Jeremias, Schierwagen, Robert, Körner, Christian, Granzow, Michaela, Huss, Sebastian, Mazar, Irela Gretchen Reza, Weber, Susanne, van den Ven, Peter F M, Pieper-Fürst, Ursula, Fürst, Dieter O, Nattermann, Jacob, Lammert, Frank, Sauerbruch, Tilman, Trebicka, Jonel

“…Hepatic myofibroblasts (MFB) show increased proliferation, migration and collagen production, which are crucial for hepatic fibrogenesis. Atorvastatin…”
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Molecular basis of F-actin regulation and sarcomere assembly via myotilin by Kostan, Julius, Pavšič, Miha, Puž, Vid, Schwarz, Thomas C, Drepper, Friedel, Molt, Sibylle, Graewert, Melissa Ann, Schreiner, Claudia, Sajko, Sara, van der Ven, Peter F M, Onipe, Adekunle, Svergun, Dmitri I, Warscheid, Bettina, Konrat, Robert, Fürst, Dieter O, Lenarčič, Brigita, Djinović-Carugo, Kristina

“…Sarcomeres, the basic contractile units of striated muscle cells, contain arrays of thin (actin) and thick (myosin) filaments that slide past each other during…”
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Translocation of molecular chaperones to the titin springs is common in skeletal myopathy patients and affects sarcomere function by Unger, Andreas, Beckendorf, Lisa, Böhme, Pierre, Kley, Rudolf, von Frieling-Salewsky, Marion, Lochmüller, Hanns, Schröder, Rolf, Fürst, Dieter O, Vorgerd, Matthias, Linke, Wolfgang A

“…Myopathies encompass a wide variety of acquired and hereditary disorders. The pathomechanisms include structural and functional changes affecting, e.g.,…”
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Breaking sarcomeres by in vitro exercise by Orfanos, Zacharias, Gödderz, Markus P. O., Soroka, Ekaterina, Gödderz, Tobias, Rumyantseva, Anastasia, van der Ven, Peter F. M., Hawke, Thomas J., Fürst, Dieter O.

“…Eccentric exercise leads to focal disruptions in the myofibrils, referred to as “lesions”. These structures are thought to contribute to the post-exercise…”
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Mutations in the N-terminal Actin-Binding Domain of Filamin C Cause a Distal Myopathy by DUFF, Rachael M, TAY, Valerie, SONG, Jaeguen, DJINOVIC-CARUGO, Kristina, PENTTILÄ, Sini, RAHEEM, Olayinka, REARDON, Katrina, MALANDRINI, Alessandro, GAMBELLI, Simona, VILLANOVA, Marcello, NOWAK, Kristen J, WILLIAMS, David R, HACKMAN, Peter, LANDERS, John E, BROWN, Robert H, UDD, Bjarne, LAING, Nigel G, RAVENSCROFT, Gianina, MCLEAN, Catriona, KENNEDY, Paul, STEINBACH, Alina, SCHÖFFLER, Wiebke, VAN DER VEN, Peter F. M, FÜRST, Dieter O

“…Linkage analysis of the dominant distal myopathy we previously identified in a large Australian family demonstrated one significant linkage region located on…”
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246th ENMC International Workshop: Protein aggregate myopathies 24–26 May 2019, Hoofddorp, The Netherlands by Olivé, Montse, Winter, Lilli, Fürst, Dieter O., Schröder, Rolf, Behin, Anthony, Breukel, Alexandra, Brumhard, Matthias, Bryson-Richardson, Robert, Claeys, Kristl, Ferreiro, Ana, Fürst, Dieter, Goebel, Hans H., Gupta, Vandana, Kley, Rudolf, Mankodi, Ami, Noguchi, Satoru, Oldfors, Anders, Olivé, Montse, Schröder, Rolf, Selcen, Duygu, Timmerman, Vincent, Udd, Bjarne, Walter, Maggie, Weihl, Conrad, Wiche, Gerhard, Winter, Lilly

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Beyond the sarcomere: CSRP3 mutations cause hypertrophic cardiomyopathy by Geier, Christian, Gehmlich, Katja, Ehler, Elisabeth, Hassfeld, Sabine, Perrot, Andreas, Hayess, Katrin, Cardim, Nuno, Wenzel, Katrin, Erdmann, Bettina, Krackhardt, Florian, Posch, Maximilian G., Bublak, Angelika, Nägele, Herbert, Scheffold, Thomas, Dietz, Rainer, Chien, Kenneth R., Spuler, Simone, Fürst, Dieter O., Nürnberg, Peter, Özcelik, Cemil

“…Hypertrophic cardiomyopathy (HCM) is a frequent genetic cardiac disease and the most common cause of sudden cardiac death in young individuals. Most of the…”
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A Mutation in the Dimerization Domain of Filamin C Causes a Novel Type of Autosomal Dominant Myofibrillar Myopathy by Vorgerd, Matthias, van der Ven, Peter F.M., Bruchertseifer, Vera, Löwe, Thomas, Kley, Rudolf A., Schröder, Rolf, Lochmüller, Hanns, Himmel, Mirko, Koehler, Katrin, Fürst, Dieter O., Huebner, Angela

“…Myofibrillar myopathy (MFM) is a human disease that is characterized by focal myofibrillar destruction and pathological cytoplasmic protein aggregations. In an…”
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Homozygous expression of the myofibrillar myopathy-associated p.W2710X filamin C variant reveals major pathomechanisms of sarcomeric lesion formation by Schuld, Julia, Orfanos, Zacharias, Chevessier, Frédéric, Eggers, Britta, Heil, Lorena, Uszkoreit, Julian, Unger, Andreas, Kirfel, Gregor, van der Ven, Peter F. M, Marcus, Katrin, Linke, Wolfgang A, Clemen, Christoph S, Schröder, Rolf, Fürst, Dieter O

“…Abstract Filamin C (FLNc) is mainly expressed in striated muscle cells where it localizes to Z-discs, myotendinous junctions and intercalated discs. Recent…”
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Novel Filamin C Myofibrillar Myopathy Variants Cause Different Pathomechanisms and Alterations in Protein Quality Systems by Sellung, Dominik, Heil, Lorena, Daya, Nassam, Jacobsen, Frank, Mertens-Rill, Janine, Zhuge, Heidi, Döring, Kristina, Piran, Misagh, Milting, Hendrik, Unger, Andreas, Linke, Wolfgang A, Kley, Rudi, Preusse, Corinna, Roos, Andreas, Fürst, Dieter O, Ven, Peter F M van der, Vorgerd, Matthias

“…Myofibrillar myopathies (MFM) are a group of chronic muscle diseases pathophysiologically characterized by accumulation of protein aggregates and structural…”
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Synaptopodin-2 Isoforms Have Specific Binding Partners and Display Distinct, Muscle Cell Type-Specific Expression Patterns by Lohanadan, Keerthika, Assent, Marvin, Linnemann, Anja, Schuld, Julia, Heukamp, Lukas C, Krause, Karsten, Vorgerd, Matthias, Reimann, Jens, Schänzer, Anne, Kirfel, Gregor, Fürst, Dieter O, Van der Ven, Peter F M

“…Synaptopodin-2 (SYNPO2) is a protein associated with the Z-disc in striated muscle cells. It interacts with α-actinin and filamin C, playing a role in Z-disc…”
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Mutations in the human muscle LIM protein gene in families with hypertrophic cardiomyopathy by GEIER, Christian, PERROT, Andreas, FÜRST, Dieter O, VORNWALD, Arnold, VON HODENBERG, Eberhard, NÜRNBERG, Peter, SCHEFFOLD, Thomas, DIETZ, Rainer, OSTERZIEL, Karl Josef, ÖZCELIK, Cemil, BINNER, Priska, COUNSELL, Damian, HOFFMANN, Katrin, PILZ, Bernhard, MARTINIAK, Yvonne, GEHMLICH, Katja, VAN DER VEN, Peter F. M

“…Muscle LIM protein (MLP) is an essential nuclear regulator of myogenic differentiation. Additionally, it may act as an integrator of protein assembly of the…”
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