2024

Umathum V., A. Weber, D. Amsel, I. Alexopoulos, C. Becker, A. Roth, A. Günther, C. Selignow, N. Ritschel, A. Nishimura, A. Schaiter, A. Németh, P.F.M. van der Ven, T. Acker, A. Schänzer. Distribution of ferritin complex in the adult brain and altered composition in neuroferritinopathy due to a novel variant in the ferritin heavy chain gene FTH1 (c.409_410del; p.H137Lfs*4). Brain Pathol. 2024;34:e13176. doi: 10.1111/bpa.13176

Lohanadan, K.; M. Assent, A. Linnemann, J. Schuld, L.C. Heukamp, K. Krause, M. Vorgerd, J. Reimann, A. Schänzer, G. Kirfel, D.O. Fürst, P.F.M. van der Ven. Synaptopodin-2 Isoforms Have Specific Binding Partners and Display Distinct, Muscle Cell Type-Specific Expression Patterns. Cells 2024;13:85. doi: 10.3390/cells13010085

2023

Roos A., P.F.M. van der Ven, H. Alrohaif, H. Kölbel, L. Heil, A. Della Marina, J. Weis, M. Aßent, S. Beck-Wödl, R. Barresi, A. Töpf, K. O'Connor, A. Sickmann, N. Kohlschmidt, M. El Gizouli, N. Meyer, N. Daya, V. Grande, K. Bois, F.J. Kaiser, M. Vorgerd, C. Schröder, U. Schara-Schmidt, A. Gangfuss, T. Evangelista, L. Röbisch, A. Hentschel, A. Grüneboom, D.O. Fürst DO, A. Kuechler, A. Tzschach, C. Depienne, H. Lochmüller. Bi-allelic variants of FILIP1 cause congenital myopathy, dysmorphism and neurological defects. Brain 2023;146:4200-4216. doi: 10.1093/brain/awad152

Sellung, D., L. Heil, N. Daya, F. Jacobsen, J. Mertens-Rill, H. Zhuge, K. Döring, A. Unger, R.A. Kley, C. Preusse, A. Roos , D.O. Fürst, P.F.M. van der Ven, M. Vorgerd. Novel filamin C myofibrillar myopathy variants cause different pathomechanisms and alterations in protein quality systems. Cells 2023;12:1321 doi.org/10.3390/cells12091321

Grande, V., J. Schuld, P.F.M. van der Ven, O. Gruss, D.O. Fürst. Filamin-A-interacting protein 1 (FILIP1) is a dual compartment protein linking myofibrils and microtubules during myogenic differentiation and upon mechanical stress. Cell Tiss. Res. 2023;393: 133-147 doi: 10.1007/s00441-023-03776-4

Schneider S., A. Kovacevic, M. Mayer, A.K. Dicke, L. Arévalo, S.A. Koser, J.N. Hansen, S. Young, C. Brenker, S. Kliesch, D. Wachten, G. Kirfel, T. Strünker, F. Tüttelmann, H. Schorle. Cylicins are a structural component of the sperm calyx being indispensable for male fertility in mice and human.Elife. 2023;12:RP86100. doi: 10.7554/eLife.86100 

Tilic E., T. Herkenrath, G. Kirfel, T. Bartolomaeus. The cellular 3D printer of a marine bristle worm-chaetogenesis in Platynereis dumerilii (Audouin & Milne Edwards, 1834) (Annelida). Cell Tissue Res. 2023;391:305-322. doi: 10.1007/s00441-022-03731-9

Merges G.E., L. Arévalo, A. Kovacevic, K. Lohanadan, D.G. de Rooij, C. Simon, M. Jokwitz, W. Witke, H. Schorle. Actl7b deficiency leads to mislocalization of LC8 type dynein light chains and disruption of murine spermatogenesis. Development 2023;150:dev201593. doi: 10.1242/dev.201593.

2022

Krause K., B. Eggers, J. Uszkoreit, S. Eulitz, R. Rehmann, A.K. Güttsches, A. Schreiner, P.F.M. van der Ven, D.O. Fürst, K. Marcus, M. Vorgerd, R.A. Kley. Target formation in muscle fibres indicates reinnervation - A proteomic study in muscle samples from peripheral neuropathies. Neuropathol Appl Neurobiol. 2022; e12853. doi: 10.1111/nan.12853.

Elsnicova B, D. Hornikova, V. Tibenska, D. Kolar, T. Tlapakova, B. Schmid, M. Mallek, B. Eggers, U. Schlötzer-Schrehardt, V. Peeva, C. Berwanger, B. Eberhard, H. Durmuş, D. Schultheis, C. Holtzhausen, K. Schork, K. Marcus, J. Jordan, T. Lücke, P.F.M. van der Ven, R. Schröder, C.S. Clemen, J.M. Zurmanova. Desmin Knock-Out Cardiomyopathy: A Heart on the Verge of Metabolic Crisis. Int J Mol Sci. 2022; 23:12020. doi: 10.3390/ijms231912020

Gangfuß A, A. Hentschel, L. Heil, M. Gonzalez, A. Schönecker, C. Depienne, A. Nishimura, D. Zengeler, N. Kohlschmidt, A. Sickmann, U. Schara-Schmidt, D.O. Fürst, P.F.M. van der Ven, A. Hahn, A. Roos, A. Schänzer. Proteomic and morphological insights and clinical presentation of two young patients with novel mutations of BVES (POPDC1). Mol Genet Metab. 2022;136:226-237. doi: 10.1016/j.ymgme.2022.05.005.  
Free download up to July 21, 2022 https://authors.elsevier.com/c/1fAOP3vNNNfLOn

Merges GE, J. Meier, S. Schneider, A. Kruse, A.C. Fröbius, G. Kirfel, K. Steger, L. Arévalo, H. Schorle. Loss of Prm1 leads to defective chromatin protamination, impaired PRM2 processing, reduced sperm motility and subfertility in male mice. Development. 2022;149:dev200330. doi: 10.1242/dev.200330.

Umer N., S. Phadke, F. Shakeri, L. Arévalo, K. Lohanadan, G. Kirfel, M. Sylvester, A. Buness, H. Schorle.PFN4 is required for manchette development and acrosome biogenesis during mouse spermiogenesis. Development. 2022;149:dev200499. doi: 10.1242/dev.200499

2021

Lohanadan K., S. Molt, F. Dierck, P.F.M. van der Ven, N. Frey, J. Höhfeld, D.O. Fürst. Isoform-specific functions of synaptopodin-2 variants in cytoskeleton stabilization and autophagy regulation in muscle under mechanical stress. Exp Cell Res. 2021; 112865. doi: 10.1016/j.yexcr.2021.112865.

Höhfeld J., T. Benzing, W. Bloch, D.O. Fürst, S. Gehlert, M. Hesse, B. Hoffmann, T. Hoppe, P.F. Huesgen, M. Köhn, W. Kolanus, R. Merkel, C.M. Niessen, W. Pokrzywa, M.M. Rinschen, D. Wachten, B. Warscheid. Maintaining proteostasis under mechanical stress. EMBO Rep. 2021:e52507. doi: 10.15252/embr.202152507.

Kley R.A., Y. Leber, B. Schrank, H. Zhuge, Z. Orfanos, J. Kostan, A. Onipe, D. Sellung, A.K. Güttsches, B. Eggers, F. Jacobsen, W. Kress, K. Marcus, K. Djinovic-Carugo, P.F.M. van der Ven, D.O. Fürst, M. Vorgerd. FLNC-associated myofibrillar myopathy: new clinical, functional, and proteomic data. Neurol Genet. 2021; 7:e590. doi: 10.1212/NXG.0000000000000590.

Popov S.V., E.S. Prokudina, A.V. Mukhomedzyanov, N.V. Naryzhnaya, H. Ma, J.M. Zurmanova, P.F.M. van der Ven, L.N. Maslov. Cardioprotective and vasoprotective effects of corticotropin-releasing hormone and urocortins: receptors and signaling. J Cardiovasc Pharmacol Ther. 2021; doi: 10.1177/1074248420985301.

Kimura K., A. Ooms, K. Graf-Riesen, M. Kuppusamy, A. Unger, J. Schuld, J. Daerr, A. Lother, C. Geisen, L. Hein, S. Takahashi, G. Li, W. Röll, W. Bloch, P.F.M. van der Ven, W.A. Linke, S.M. Wu, P.F. Huesgen, J. Höhfeld, D.O. Fürst, B.K. Fleischmann, M. Hesse. Overexpression of human BAG3P209L in mice causes restrictive cardiomyopathy. Nat Commun. 2021;12:3575. doi: 10.1038/s41467-021-23858-7.

Kostan J., M. Pavšič, V. Puž, T.C. Schwarz, F. Drepper, S. Molt, M.A. Graewert, C. Schreiner, S. Sajko, P.F.M. van der Ven, A. Onipe, D.I. Svergun, B. Warscheid, R. Konrat, D.O. Fürst, B. Lenarčič, K. Djinović-Carugo. Molecular basis of F-actin regulation and sarcomere assembly via myotilin. PLoS Biol. 2021; 19:e3001148. doi: 10.1371/journal.pbio.3001148.

Schänzer A, E. Schumann, D. Zengeler, L. Gulatz, G. Maroli, U. Ahting, A. Sprengel, S. Gräf, A. Hahn, C. Jux, T. Acker, D.O. Fürst, S. Rupp, J. Schuld, P.F.M. van der Ven. The p.Ala2430Val mutation in filamin C causes a "hypertrophic myofibrillar cardiomyopathy". J Muscle Res Cell Motil. 2021;42:381-397. doi: 10.1007/s10974-021-09601-1.

Olivé M., L. Winter, D.O. Fürst, R. Schröder; ENMC workshop study group. 246th ENMC International Workshop: Protein aggregate myopathies 24-26 May 2019, Hoofddorp, The Netherlands. Neuromuscul Disord. 2021; 31:158-166. doi: 10.1016/j.nmd.2020.11.003.

N. Umer, L. Arévalo, S. Phadke, K. Lohanadan, G. Kirfel, D. Sons, D. Sofia, W. Witke, H. Schorle. Loss of Profilin3 Impairs Spermiogenesis by Affecting Acrosome Biogenesis, Autophagy, Manchette Development and Mitochondrial Organization. Front Cell Dev Biol. 2021 Nov 4;9:749559. doi: 10.3389/fcell.2021.749559.

2020

Mao Y., R. Schneider, P.F.M. van der Ven, M. Assent, K. Lohanadan, V. Klämbt, F. Buerger, T.M. Kitzler, K. Deutsch, M. Nakayama, A.J. Majmundar, N. Mann, T. Hermle, A.C. Onuchic-Whitford, W. Zhou, N.N. Margam, R. Duncan, J. Marquez, M. Khokha, H.M. Fathy, J.A. Kari, S. El Desoky, L.A. Eid, H.S. Awad, M. Al-Saffar, S. Mane, R.P. Lifton, D.O. Fürst, S. Shril, F. Hildebrandt. Recessive Mutations in SYNPO2 as a Candidate of Monogenic Nephrotic Syndrome. Kidney Int Rep. 2020; 6:472-483. doi: 10.1016/j.ekir.2020.10.040.

Detection of endogenous lipids in chicken feathers distinct from preen gland constituents.
Zeisler-Diehl V., E.A.A. Al-Khutabi, G. Kirfel, L. Schreiber, G. van Echten-Deckert, V. Herzog. Protoplasma. 2020; 257:1709-1724. doi: 10.1007/s00709-020-01544-7.

Schuld, J., Z. Orfanos, F. Chevessier, B. Eggers, L. Heil, J. Uszkoreit, A. Unger, G. Kirfel, P.F.M. van der Ven, K. Marcus, W.A. Linke, C.S. Clemen, R. Schröder, D.O. Fürst Homozygous expression of the myofibrillar myopathy-associated p.W2710X filamin C variant reveals major pathomechanisms of sarcomeric lesion formation. Acta Neuropathol Commun. 2020; 8(1):154. doi: 10.1186/s40478-020-01001-9. PMID: 32887649

Kölbel H, A. Roos, P.F.M. van der Ven, T. Evangelista, K. Nolte, K. Johnson, A. Töpf, M. Wilson, W. Kress, A. Sickmann, V. Straub, L. Kollipara, J. Weis, D.O. Fürst, U. Schara. First clinical and myopathological description of a myofibrillar myopathy with congenital onset and homozygous mutation in FLNC. Hum Mutat. 2020; 41:1600-1614. doi: 10.1002/humu.24062. Epub 2020 Jul 11. PMID: 32516863

Reimann L, A.N. Schwäble, A.L. Fricke, W.W.D. Mühlhäuser, Y. Leber, K. Lohanadan, M.G. Puchinger, S. Schäuble, E. Faessler, H. Wiese, C. Reichenbach, B. Knapp, C.D. Peikert, F. Drepper, U. Hahn, C. Kreutz, P.F.M. van der Ven, G. Radziwill, K. Djinović-Carugo, D.O. Fürst, Warscheid B. Phosphoproteomics identifies dual-site phosphorylation in an extended basophilic motif regulating FILIP1-mediated degradation of filamin-C. Commun Biol. 2020; 3:253. doi: 10.1038/s42003-020-0982-5. PMID: 32444788

2019

 Collier, M.P., T.R. Alderson, C.P. de Villiers, D. Nicholls, H.Y. Gastall, T.M. Allison, M. T. Degiacomi,H. Jiang,G. Mlynek, D.O. Fürst,P.F.M. van der Ven, K. Djinovic-Carugo,A.J. Baldwin,H. Watkins, K. Gehmlich, J.L.P. Benesch. HspB1 phosphorylation regulates its intramolecular dynamics and mechanosensitive molecular chaperone interaction with filamin C. Science Advances 2019; 5:5, eaav8421. doi: 10.1126/sciadv.aav8421.

 Klein S., F. Frohn, F. Magdaleno, C. Reker-Smit, R. Schierwagen, I. Schierwagen, F.E. Uschner, F. van Dijk, D.O. Fürst, S. Djudjaj, P. Boor, K. Poelstra, L. Beljaars, J. Trebicka. Rho-kinase inhibitor coupled to peptide-modified albumin carrier reduces portal pressure and increases renal perfusion in cirrhotic rats.Sci. Rep. 2019;9:2256. doi: 10.1038/s41598-019-38678-5.

2017

Unger A., L. Beckendorf, P. Böhme, R. Kley, M. von Frieling-Salewsky, H. Lochmüller, R. Schröder, D.O. Fürst, M. Vorgerd, W.A. Linke. Translocation of molecular chaperones to the titin springs is common in skeletal myopathy patients and affects sarcomere function. Acta Neuropathol. Commun. 2017; 5:72. doi: 10.1186/s40478-017-0474-0

Dierck F., C. Kuhn, C. Rohr, S. Hille, J. Braune, S. Sossalla, S. Molt, P.F.M. van der Ven, D.O. Fürst, N. Frey (2017) The novel cardiac z-disc protein CEFIP regulates cardiomyocyte hypertrophy by modulating calcineurin signaling. J Biol Chem. 292:15180-15191.doi: 10.1074/jbc.M117.786764

 Reimann L., H. Wiese, Y. Leber, A.N. Schwäble, A.L. Fricke, A. Rohland, B. Knapp, C.D. Peikert, F. Drepper, P.F.M. van der Ven, G. Radziwill, D.O. Fürst, B. Warscheid. (2017) Myofibrillar Z-discs are a protein phosphorylation hot spot with PKC α modulating protein dynamics.Mol Cell Proteomics pii: mcp.M116.065425. doi: 10.1074/mcp.M116.065425[Epub ahead of print]

2016

Kathage B., S. Gehlert, A. Ulbricht, L. Lüdecke, V.E. Tapia, Z. Orfanos, D. Wenzel, W. Bloch, R. Volkmer, B.K. Fleischmann, D.O. Fürst, J. Höhfeld. (2016) The cochaperone BAG3 coordinates protein synthesis and autophagy under mechanical strain through spatial regulation of mTORC1.Biochim. Biophys. Acta. 1864:62-75. doi: 10.1016/j.bbamcr.2016.10.007.

Kebir S., Z. Orfanos, J. Schuld, M. Linhart, C. Lamberz, P.F.M. van der Ven, J. Schrickel, G. Kirfel, D.O. Fürst, R. Meyer. (2016) Sarcomeric lesions and remodeling proximal to intercalated disks in overload-induced cardiac hypertrophy.Exp. Cell Res. 348:95-105. doi: 10.1016/j.yexcr.2016.09.008 .

Avila-Smirnow D, L. Gueneau, S. Batonnet-Pichon, F. Delort, H.M. Bécane, K. Claeys, M. Beuvin, B. Goudeau, J.P. Jais, I. Nelson, P. Richard, R. Ben Yaou, N.B. Romero, K. Wahbi, S. Mathis, T. Voit, D. Furst, P. van der Ven, R. Gil, P. Vicart, M. Fardeau, G. Bonne, A. Behin. (2016) Cardiac arrhythmia and late-onset muscle weakness caused by a myofibrillar myopathy with unusual histopathological features due to a novel missense mutation in FLNC.Rev. Neurol. 172:594-606. doi: 10.1016/j.neurol.2016.07.017 .

Hedberg-Oldfors C., N. Darin, M. Olsson Engman, Z. Orfanos, C. Thomsen, P.F.M. van der Ven, A. Oldfors. (2016) A new early-onset neuromuscular disorder associated with kyphoscoliosis peptidase (KY) deficiency.Eur. J. Hum. Genet. 24:1771-1777. doi: 10.1038/ejhg.2016.98 .

Straussberg R, G. Schottmann, M. Sadeh, E. Gill, F. Seifert, A. Halevy, K. Qassem, J. Rendu, P.F.M. van der Ven, W. Stenzel, M. Schuelke. (2016) Kyphoscoliosis peptidase (KY) mutation causes a novel congenital myopathy with core targetoid defects.Acta Neuropathol. 132:475-478. doi: 10.1007/s00401-016-1602-9 .

Leber Y, A.A. Ruparelia, G. Kirfel, P.F.M. van der Ven, B. Hoffmann, R. Merkel, R.J. Bryson-Richardson, D.O. Fürst. (2016) Filamin C is a highly dynamic protein associated with fast repair of myofibrillar microdamage.Hum. Mol. Genet. [Epub ahead of print]. pii: ddw135.

Orfanos, Z., M. P. O. Gödderz, E. Soroka, T. Gödderz, A. Rumyantseva, P. F. M. van der Ven, T. J. Hawke, D. O. Fürst (2016) Breaking sarcomeres by in vitro exercise. Sci. Rep. 6, 19614. doi: 10.1038/srep19614.

Maerkens, A., M. Olivé, A. Schreiner, S. Feldkirchner, J. Schessl, J. Uszkoreit, K. Barkovits, A.K. Güttsches, V. Theis, M. Eisenacher, M. Tegenthoff, L. G. Goldfarb, R. Schröder, B. Schoser, P. F. M. van der Ven, D. O. Fürst, M. Vorgerd, K. Marcus, R. A. Kley (2016) New insights into the protein aggregation pathology in myotilinopathy by combined proteomic and immunolocalization analyses. Acta Neuropathol. Commun. 4:549. doi:10.1186/s40478-016-0280-0

2015

Görtzen, J.,R. Schierwagen,J. Bierwolf.,S. Klein.,F. E. Uschner.,P. F. van der Ven,D. O. Fürst,C. P. Strassburg,W. Laleman, J.-M.Pollok, J.Trebicka (2015) Interplay of Matrix Stiffness and c-SRC in Hepatic Fibrosis.Front. Physiol. 6:46.doi: 10.3389/fphys.2015.00359

Chevessier, F., J. Schuld, Z. Orfanos, A.-C. Plank, L. Wolf, A. Maerkens, A. Unger, U. Schlötzer-Schrehardt, R. A. Kley, S. von Hörsten, K. Marcus, W. A. Linke, M. Vorgerd, P. F. M. van der Ven, D. O. Fürst, R. Schröder (2015) Myofibrillar instability exacerbated by acute exercise in filaminopathy.Hum. Mol. Genet. 24:7207-7220. doi: 10.1093/hmg/ddv421.

Uschner, F.E., G. Ranabhat, S.S. Choi, M. Granzow, S. Klein, R. Schierwagen, E. Raskopf, S. Gautsch, P.F.M. van der Ven, D.O. Fürst, C.P. Strassburg, T. Sauerbruch, A. Mae Diehl, J. Trebicka (2015) Statins activate the canonical hedgehog-signaling and aggravate non-cirrhotic portal hypertension, but inhibit the non-canonical hedgehog signaling and cirrhotic portal hypertension.Sci. Rep. 5:14573. doi: 10.1038/srep14573. doi: 10.1038/srep14573

Al-Sajee, D., A. A. Nissar, S. K. Coleman, I. A. Rebalka, A. Chiang, R. Wathra, P. F. M. van der Ven, Z. Orfanos, and T. J. Hawke (2015). Xin-deficient mice display myopathy, impaired contractility, attenuated muscle repair and altered satellite cell functionality. Acta Physiol. 214:248–260. doi:10.1111/apha.12455.

Bührdel, J. B., S. Hirth, M. Keßler, S. Westphal, M. Forster, L. Manta, G. Wiche, B. Schoser, J. Schessl, R. Schröder, C. S. Clemen, L. Eichinger, D. O. Fürst, P. F. M. van der Ven, W. Rottbauer, and S. Just (2015). In vivo characterization of human myofibrillar myopathy genes in zebrafish. Biochem. Biophys. Res. Commun. 461:217–223. doi:10.1016/j.bbrc.2015.03.149.

Olivé, M., S. Abdul-Hussein, A. Oldfors, J. González-Costello, P. F. M. van der Ven, D. O. Fürst, L. Gonzalez-Mera, D. Moreno, B. Torrejón-Escribano, J. Alió, A. Pou, I. Ferrer, and H. Tajsharghi (2015). New cardiac and skeletal protein aggregate myopathy associated with combined MuRF1 and MuRF3 mutations. Hum. Mol. Genet. 24:3638-3650. doi:10.1093/hmg/ddv108.

2014

Granzow, M., R. Schierwagen, S. Klein, B. Kowallick, S. Huss, M. Linhart, I. G. Reza Mazar, J. Görtzen, A. Vogt, F. A. Schildberg, M.-A. Gonzalez-Carmona, A. Wojtalla, B. Krämer, J. Nattermann, S. V. Siegmund, N. Werner, D. O. Fürst, W. Laleman, P. Knolle, V. H. Shah, T. Sauerbruch, J. Trebicka (2014). Angiotensin-II type 1 receptor-mediated Janus-kinase-2-activation induces fibrosis. Hepatology 60, 334-348

Gurniak, C. B., F. Chevessier, M. Jokwitz, F. Jönsson, E. Perlas, H. Richter, G. Matern, P. P. Boyl, C. Chaponnier, D. O. Fürst, R. Schröder, W. Witke (2014). Severe protein aggregate myopathy in a knockout mouse model points to an essential role of cofilin2 in sarcomeric actin exchange and muscle maintenance. Eur. J. Cell Biol. 93, 252-266

Hossain, M. M., D. Tesfaye, D. Salilew-Wondim, E. Held, M. J. Pröll, F. Rings, G. Kirfel, C. Looft, E. Tholen, J. Uddin, K. Schellander, M. Hoelker (2014). Massive deregulation of miRNAs from nuclear reprogramming errors during trophoblast differentiation for placentogenesis in cloned pregnancy. BMC Genomics 15:43. doi: 10.1186/1471-2164-15-43

Molt S., J. B. Bührdel, S. Yakovlev, P. Schein, Z. Orfanos, G. Kirfel, L. Winter, G. Wiche, P.F.M. van der Ven, W. Rottbauer, S. Just, A. M. Belkin, D. O. Fürst (2014). Aciculin interacts with filamin C and Xin and is essential for myofibril assembly, remodeling and maintenance. J. Cell Sci. 127, 3578-3592

2013

Abdul-Hussein, S., P. F. M. van der Ven, H. Tajsharghi (2013). Expression profiles of muscle disease-associated genes and their isoforms during differentiation of cultured human skeletal muscle cells. BMC Musculoskelet. Disord. 13, 262, doi: 10.1186/1471-2474-13-262

Eulitz S, F. Sauer, M. C. Pelissier, P. Boisguerin, S. Molt, J. Schuld, Z. Orfanos, R. A. Kley, R. Volkmer, M. Wilmanns, G. Kirfel, P. F. M. van der Ven, D. O. Fürst (2013). Identification of Xin-repeat proteins as novel ligands of the SH3 domains of nebulin and nebulette and analysis of their interaction during myofibril formation and remodeling. Mol. Biol. Cell. 24, 3215-3226

Fürst D. O., L. G. Goldfarb, R. A. Kley, M. Vorgerd, M. Olivé, P. F. M. van der Ven (2013). Filamin C-related myopathies: pathology and mechanisms. A. Neuropathol. 125, 33-46

Kley, R. A., A. Maerkens, Y. Leber, V. Theis, A. Schreiner, P. F. M. van der Ven, J. Uszkoreit, C. Stephan, S. Eulitz, N. Euler, J. Kirschner, K. Mueller, H. E. Meyer, M. Tegenthoff, D. O. Fürst, M. Vorgerd, T. Müller, K. Marcus (2013). A combined laser microdissection and mass spectrometry approach reveals new disease relevant proteins accumulating in aggregates of filaminopathy patients. Mol. Cell. Proteom. 12, 215-227

Kley, R. A., P. F. M. van der Ven, M. Olivé, J. Höhfeld, L. G. Goldfarb, D. O. Fürst, M. Vorgerd (2013). Impairment of protein degradation in myofibrillar myopathy caused by FLNC/ filamin C mutations. Autophagy 9, 422-423

Linnemann A., P. Vakeel, E. Bezerra, Z. Orfanos, K. Djinović-Carugo, P. F. M. van der Ven, G. Kirfel, D. O. Fürst (2013). Myopodin is an F-actin bundling protein with multiple independent actin-binding regions. J. Muscle Res. Cell Motil. 34, 61-69

Maerkens A., R. A. Kley, M. Olivé, V. Theis, P. F. M. van der Ven, J. Reimann, H. Milting, A. Schreiner, J. Uszkoreit, M. Eisenacher, K. Barkovits, A. K. Güttsches, J. Tonillo, K. Kuhlmann, H. E. Meyer, R. Schröder, M. Tegenthoff, D. O. Fürst, T. Müller, L. G. Goldfarb, M. Vorgerd, K. Marcus (2013). Differential proteomic analysis of abnormal intramyoplasmic aggregates in desminopathy. J. Proteomics 90, 14-27

Nilsson M. I., A. A. Nissar, D. Al-Sajee, M. A. Tarnopolsky, G. Parise, B. Lach, D. O. Fürst, P. F. M. van der Ven, R. A. Kley, T. J. Hawke (2013). Xin is a marker of skeletal muscle damage severity in myopathies. Am. J. Pathol. 183, 1703-1709

Sedaghat, A., J. M. Sinning, K. Paul, G. Kirfel, G. Nickening, N. Werner (2013). First in vitro and in vivo results of an anti-human CD133-antibody coated coronary stent in the porcine model. Clin. Res. Cardiol. 102, 413-25

Ulbricht A., F. J. Eppler, V. E. Tapia, P. F. M. van der Ven, N. Hampe, N. Hersch, P. Vakeel, D. Stadel, A. Haas, P. Saftig, C. Behrends, D. O. Fürst, R. Volkmer, B. Hoffmann, W. Kolanus, J. Höhfeld (2013). Cellular mechanotransduction relies on tension-induced and chaperone-assisted autophagy. Curr. Biol. 23, 430-435

2012

Jönsson F., C. B. Gurniak, B. Fleischer, G. Kirfel, W. Witke (2012). Immunological responses and actin dynamics in macrophages are controlled by N-cofilin but are independent from ADF. PLoS One 7:e36034. doi: 10.1371/journal.pone.0036034

Klein S., J. Klosel, R. Schierwagen, C. Korner, M. Granzow, S. Huss, I. G. Mazar, S. Weber, P. F. M. van der Ven, U. Pieper-Fürst, D. O. Fürst, J. Nattermann, F. Lammert, T. Sauerbruch, J. Trebicka (2012). Atorvastatin inhibits proliferation and apoptosis, but induces senescence in hepatic myofibroblasts and thereby attenuates hepatic fibrosis in rats. Lab. Invest. 92, 1440-1450

Kley R. A., P. Serdaroglu-Oflazer, Y. Leber, Z. Odgerel, P. F. M. van der Ven, M. Olivé, I. Ferrer, A. Onipe, M. Mihaylov, J.M. Bilbao, H.S. Lee, J. Höhfeld, K. Djinović-Carugo, K. Kong, M. Tegenthoff, S. A. Peters, W. Stenzel, M. Vorgerd, L. G. Goldfarb, D. O. Fürst (2012). Pathophysiology of protein aggregation and extended phenotyping in filaminopathy. Brain 135, 2642-2660

Lamsoul I., M. Erard, P.F.M. van der Ven, P.G. Lutz (2012). Filamins but not Janus kinases are substrates of the ASB2alpha cullin-ring E3 ubiquitin Ligase in Hematopoietic Cells. PLoS ONE 7:e43798. doi: 10.1371/journal.pone.0043798

Nissar, A. A., B. Zemanek, R. Labatia, D. J. Atkinson, P. F. M. van der Ven, D. O. Fürst, T. J. Hawke (2012). Skeletal muscle regeneration is delayed by reduction in Xin expression: consequence of impaired satellite cell activation? Am. J. Physiol. Cell. Physiol. 302:C220-C227

 Otten, C., P. F. M. van der Ven, I. Lewrenz, S. Paul, A. Steinhagen, E. Busch-Nentwich, J. Eichhorst, B. Wiesner, D. Stemple, U. Strähle, D. O. Fürst, S. Abdelilah-Seyfried (2012). Xirp proteins mark injured skeletal muscle in zebrafish. PloS One 7:e31041. doi: 10.1371/journal.pone.0031041

Schneider, M., G. Kirfel, M. Berthold, M. Frentzen, F. Krause, A. Braun (2012). The impact of antimicrobial photodynamic therapy in an artificial biofilm model. Lasers Med. Sci. 27, 615–620

2011

Becher, U. M., A. Ghanem, V. Tiyerili, D. O. Fürst, G. Nickenig, C. F. H. Mueller (2011). Inhibition of leukotriene C4 action reduces oxidative stress and apoptosis in cardiomyocytes and impedes remodeling after myocardial injury. J. Mol. Cell. Cardiol. 50, 570-577

Duff, R. M., V. Tay, P. Hackman, G. Ravenscroft, C. McLean, P. Kennedy, A. Steinbach, W. Schoffler, P. F. M. van der Ven, D. O. Fürst, J. Song, K. Djinovic-Carugo, S. Penttila, O. Raheem, K. Reardon, A. Malandrini, S. Gambelli, M. Villanova, K. J. Nowak, D. R. Williams, J. E. Landers, R. H. Brown, Jr., B. Udd, N. G. Laing (2011). Mutations in the N-terminal actin-binding domain of filamin C cause a distal myopathy. Am. J. Hum. Genet. 88, 729-740

Fürst, D. O. and P. F. M. van der Ven (2011). Molekulare Analyse der Filamin C Myopathie. Jahrbuch der Deutschen Gesellschaft für Muskelkranke 2010 (R. Dengler & B. Neundörfer, Eds.).

Guergueltcheva, V., K. Peeters, J. Baets, G. C. Ceuterick-de Groote, J. J. Martin, A. Suls, V. E. De Vriendt. Mihaylova, T. Chamova, L. Almeida-Souza, E. Ydens, C. Tzekov, G. Hadjidekov, M. Gospodinova, K. Storm, E. Reyniers, S. Bichev, P. F. M. van der Ven, D. O. Fürst, V. Mitev, H. Lochmüller, V. Timmerman, I. Tournev, J. P. De Jonghe, A. Jordanova (2011). Distal myopathy with upper limb predominance caused by filamin C haploinsufficiency. Neurology 77, 2105-2114 [http://www.ncbi.nlm.nih.gov/pubmed/22131542]

Jantscheff, P., M. Schlesinger, U. Massing, J. Fritzsche, L. A. Taylor, R. Graeser, G. Kirfel, D. O. Fürst, U. Massing, G. Bendas (2011). Lysophosphatidylcholine pretreatment reduces VLA-4 and P-selectin mediated B16.F10 melanoma cell adhesion in vitro and inhibits metastasis-like lung-invasion in vivo. Mol. Cancer Ther. 10, 186-197

Schneider, M., G. Kirfel, M. Berthold, M. Frentzen, F. Krause, A. Braun (2011). The impact of antimicrobial photodynamic therapy in an artificial biofilm model. Lasers Med. Sci. 27, 615-620

Westhofen, P., M.Watzka, M.Marinova, M.Hass, G.Kirfel, J.Muller, C.G.Bevans, C.R.Muller, J.Oldenburg (2011). Human vitamin K 2,3-epoxide reductase complex subunit 1-like 1 (VKORC1L1) mediates vitamin K-dependent intracellular antioxidant function. J. Biol. Chem. 286, 15085-15094

2010

Arndt, V., N. Dick, R. Tawo, D. Wenzel, D. O. Fürst, R. Saint, B. K. Fleischmann, M. Hoch, J. Höhfeld (2010). Chaperone-assisted degradation is essential for muscle maintenance. Curr. Biol. 20, 143-148

Kesireddy, V., P. F. M. van der Ven, D. O. Fürst (2010). Multipurpose modal lentiviral vectors for RNA interference and transgene expression. Mol. Biol. Rep. 37, 2863-2870

Fürst, D. O., G. Kirfel, P. F. M. van der Ven (2010). Macromolecular assembly at its best: how to organise a muscle cell. Cell News 36, 14-18

Gehmlich K., K. Hayess, C. Legler, S. Haebel, D. Wells, P. F. M. van der Ven, E. Ehler, D. O. Fürst (2010). Ponsin interacts with Nck adapter proteins: implications for a role in cytoskeletal remodelling during differentiation of skeletal muscle cells. Eur. J. Cell Biol. 89, 351-364

Gehmlich, K, E. Ehler, A. Perrot, D. O. Fürst, C. Geier (2010). Letter to the Editor Regarding: "MLP: A Stress Sensor Goes Nuclear" by S. Gunkel, J. Heineke, D. Hilfiker-Kleiner, R. Knöll. J. Mol. Cell. Cardiol. 48, 424-425

Gerlach D., T. Kaminski, F. Pérez-Willard, G. Kirfel, V. Gieselmann, J. Kappler (2010). Nanofibers in a hyaluronan-based pericellular matrix. Matrix Biol. 29, 664-667

Linnemann, A., P. F. M. van der Ven, P. Vakeel, B. Albinus, D. Simonis, G. Bendas, J. A. Schenk, B. Micheel, R. A. Kley, D. O. Fürst (2010). The sarcomeric Z-disc component myopodin is a multiadapter protein that interacts with filamin and alpha-actinin. Eur. J. Cell Biol. 89, 681-692

Odgerel Z., L. G. Goldfarb, D. O. Fürst, P. F. M. van der Ven (2010). DNA sequencing inconsistency precluding precise molecular diagnosis of filamin myopathy. Clin. Chem. Lab. Med. 48, 1409-1414

Otten, J., P. F. M. van der Ven, P. Vakeel, S. Eulitz, G. Kirfel, O. Brandau, M. Boesl, J. Schrickel, M. Linhart, K. Hayeß, H. Milting, R. Meyer, D. O. Fürst (2010). Complete loss of murine Xin results in a mild cardiac phenotype with altered distribution of intercalated discs. Cardiovasc. Res. 85, 739-750

Pusch, A., A. Boeckenhoff, T. Glaser, T. Kaminski, G. Kirfel, M. Hans, B. Steinfarz, D. Swandulla, U. Kubitscheck, V. Gieselmann, O. Brüstle, J. Kappler (2010). CD44 and hyaluronan promote invasive growth of B35 neuroblastoma cells into the brain. Biochim. Biophys. Acta. 1803, 261-274

Thewes, V., F. Orso, R. Jäger, D. Eckert, S. Schäfer, G. Kirfel, S. Garbe, D. Taverna, H. Schorle (2010). Interference with Activator Protein-2 transcription factors leads to induction of apoptosis and an increase in chemo- and radiation-sensitivity in breast cancer cells. BMC Cancer 10, 192. doi: 10.1186/1471-2407-10-192

van der Ven, P. F. M., Z. Odgerel, D. O. Fürst, L. G. Goldfarb, S. Kono, H. Miyajima (2010). Letter to the Editor: Dominant-negative effects of a novel mutation in the filamin myopathy. Neurology 75, 2137-2138

2009

Becher, U. M., M. Breitbach, P. Sasse, S. Garbe, P. F. M. van der Ven, D. O. Fürst, B. K. Fleischmann (2009). Enrichment and terminal differentiation of muscle cells in vitro. Exp. Cell Res. 315, 2741-2451

Claeys, K. G., P. F. M. van der Ven, A. Behin, T. Stojkovic, B. Eymard, O. Dubourg, P. Laforêt, G. Faulkner, P. Richard, P. Vicart, N. B. Romero, G. Stoltenburg, B. Udd, M. Fardeau, T. Voit, D. O. Fürst (2009). Differential involvement of sarcomeric proteins in myofibrillar myopathies: A morphological and immunohistochemical study. A. Neuropathol. 117, 293-307

Gehmlich, K., C. Geier, H. Milting, D. O. Fürst, E. Ehler (2009). Back to square one: What do we know about the functions of Muscle LIM Protein in the heart? (Review). J. Musc. Res. Cell Motil. 17, 2753-2765 [http://www.ncbi.nlm.nih.gov/pubmed/19115046]

2008

Bit-Avragim, N., S. Rohr, F. Rudolph, P. van der Ven, D. Fürst, J. Eichhorst, B. Wiesner, S. Abdelilah-Seyfried (2008). Nuclear localization of the zebrafish tight junction protein nagie oko. Dev. Dyn. 237, 83-90

Geier, C., K.Gehmlich, E. Ehler, S. Hassfeld, A. Perrot, K. Hayeß, N. Cardim, K. Wenzel, B. Erdmann, F. Krackhardt, M. G. Posch, K. J. Osterziel, A. Bublak, H. Nagele, T. Scheffold, R. Dietz, K. R. Chien, S. Spuler, D. O. Fürst, P. Nürnberg, C. Özcelik (2008). Beyond the sarcomere: CSRP3 mutations cause hypertrophic cardiomyopathy. Hum. Mol. Genet. 17, 2753-2765

Wörsdörfer, P., S. Maxeiner, C. Markopoulos, G. Kirfel, V. Wulf, T. Auth, S. Urschel, J. von Maltzahn, K. Willecke (2008). Connexin expression and functional analysis of gap junctional communication in mouse embryonic stem cells. Stem Cells 26, 431-439

2007

Gehmlich, K., N. Pinotsis, K. Hayeß, P. F. M. van der Ven, H. Milting, A. El Banayosy, R. Körfer, M. Wilmanns, E. Ehler and D. O. Fürst (2007). Paxillin and ponsin interact in nascent costameres of muscle cells. J. Mol. Biol. 369, 665-682.

Löwe, T., R. A. Kley, P. F. M. van der Ven, M. Himmel, A. Huebner, M. Vorgerd and D. O. Fürst (2007). The pathomechanism of filaminopathy: altered biochemical properties explain the cellular phenotype of a protein aggregation myopathy. Hum. Mol. Genet. 16, 1351-1358.

Hawke, T. J., D. J. Atkinson, S. B. Kanatous, P. F. M. van der Ven, S. C. Goetsch and D. J. Garry (2007). Xin, an actin binding protein, is expressed within muscle satellite cells and newly regenerated skeletal muscle fibres. Am. J. Physiol. 293, C1636-C1644.

Kley, R., Hellenbroich, P. F. M. van der Ven, D. O. Fürst, A. Huebner, Heyer, Kirschner, R. Schröder, D. Fischer, Müller, K. Tolksdorf, Eger, Germing, Brodherr, Reum, M. Walter, H. Lochmüller, Ketelsen, M. Vorgerd (2007). Clinical and morphological features in filaminopathy: a newly identified subgroup of myofibrillar myopathy. Brain 130, 3250-3264.

van Echten-Deckert G., Saathoff M., Kirfel G. and Herzog V. (2007) Specific distribution of barrier-relevant ceramides in the emerging epidermis and the periderm/subperiderm during chicken embryogenesis. Eur J Cell Biol. 86:675-682.

Hartwig B, Borm B, Schneider H, Arin MJ, Kirfel G and Herzog V. (2007) Laminin-5-deficient human keratinocytes: defective adhesion results in a saltatory and inefficient mode of migration. Exp Cell Res.313:1575-87.

2006

Beatham, J., K. Gehmlich, P. F. M. van der Ven, J. Sarparanta, D. Williams, P. Underhill, C. Geier, D. O. Fürst, B. Udd and G. Blanco (2006). Constitutive upregulations in KY deficient muscles suggest early titin involvement in the mechanism of pathogenesis. Neuromusc. Disord. 16, 437-445.

Kjellgren, D., P. Stål, L. Larsson, D. O. Fürst and F. Pedrosa-Domellöf (2006). Uncoordinated expression of myosin heavy chains and myosin binding protein C isoforms in human extraocular muscles. Invest. Ophtalmol. Vis. Sci. 47, 4188-4193.

Siemes C., T. Quast, C. Kummer, S. Wehner, G. Kirfel, U. Müller & V. Herzog (2006). Keratinocytes from APP/APLP2 deficient mice are impaired in proliferation, adhesion and migration in vitro. Exp. Cell Res. 312, 1939-1949.

Van der Ven, P.F.M., E.Ehler, P.Vakeel, S.Eulitz, J.A.Schenk, H.Milting, B.Micheel, D.O.Fürst (2006). Unusual splicing events result in distinct Xin isoforms that associate differentially with filamin c and Mena/VASP. Exp.Cell Res. 312, 2154-2167

Fischer, D., C.S.Clemen, M.Olive, I.Ferrer, B.Goudeau, U.Roth, P.Badorf, M.P.Wattjes, G.Lutterbey, T.Kral, P.F.M. van der Ven, D.O.Fürst, P.Vicart, L.G.Goldfarb, M.Moza, O.Carpén, J.Reichelt, R.Schröder (2006). Different early pathogenesis in myotilinopathy compared to primary desminopathy. Neuromuscul. Disord., 16, 361-367

Cherepanova, O., A. Orlova, V.E. Galkin, P.F.M. van der Ven, D.O. Fürst, J.-P. Jin, E.H. Egelman (2006). Xin-repeats and nebulin-like repeats bind to F-actin in a similar manner. J. Mol. Biol. 356:714-723.

Raynaud F., C. Jond-Necand, A. Marcilhac, D. O. Fürst and Y. Benyamin (2006). Calpain 1-gamma filamin interaction in muscle cells: A possible in situ regulation by PKC-alpha. Int. J. Biochem. Cell Biol. 38, 404-413.

Tesfaye D, Kadanga A, Rings F, Bauch K, Jennen D, Nganvongpanit K, Hölker M, Tholen E, Ponsuksili S, Wimmers K, Montag M, Gilles M, Kirfel G, Herzog V, Schellander K. (2006). The effect of nitric oxide inhibition and temporal expression patterns of the mRNA and protein products of nitric oxide synthase genes during in vitro development of bovine pre-implantation embryos. Reprod Domest Anim. 41:501-9.

Torka R, Thuma F, Herzog V, Kirfel G. (2006) ROCK signaling mediates the adoption of different modes of migration and invasion in human mammary epithelial tumor cells.Exp Cell Res. 312:3857-71.

2005

Lange, B. M., G. Kirfel, I. Gestmann, V. Herzog & C. Gonzalez (2005). Structure and microtubule-nucleation activity of isolated Drosophila embryo centrosomes characterized by whole mount scanning and transmission electron microscopy. Histochem. Cell Biol.124, 325-334.

Ottenheijm, C.A., L.M. Heunks, T. Hafmans, P.F.M. van der Ven, C. Benoist, H. Zhou, S. Labeit, H.L. Granzier, P.N. Dekhuijzen (2005). Titin and Diaphragm Dysfunction in Chronic Obstructive Pulmonary Disease. Am J Respir Crit Care Med. 173:527-534.

Vorgerd M., P. F. M. van der Ven, V. Bruchertseifer, T. Löwe, R. A. Kley, R. Schröder, H. Lochmüller, M. Himmel, K. Koehler, D. O. Fürst und A. Huebner (2005). A mutation in the dimerization domain of filamin C causes a novel type of autosomal dominant myofibrillar myopathy. /Am. J. Hum. Genet./ 77, 297-304.

Lange, S., M. Himmel, D. Auerbach, I. Agarkova, K. Hayess, D. O. Fürst, J.-C. Perriard & E. Ehler (2005). Towards a three-dimensional model of the sarcomeric M-band based on molecular interactions. J. Mol. Biol. 345, 289-298.

Borm, B., R. Requardt, V. Herzog & G. Kirfel (2005). Membrane ruffles: indicators of disturbed cell-substrate adhesion and compartment of actin reorganisation. Exp. Cell Res. 302, 83-95.

2004

Rigort, A., J. Grünewald, V. Herzog & G. Kirfel (2004). Release of integrin macroaggregates as a mechanism of rear detachment during keratinocyte migration. Eur. J. Cell Biol. 83, 725-733.

Beatham, J., R.Romero, S.K.Townsend, T.Hacker, P.F.M. van der Ven, & G.Blanco. (2004). Filamin C interacts with the muscular dystrophy KY protein and is abnormally distributed in mouse KY deficient muscle fibres. Hum. Mol. Genet. 13, 2863-2874.

Fürst, D. O. (2004). Molekulare Physiologie des Muskels. in "Muskelkrankheiten: Diagnose und Therapie", S. Spuler & A. von Moers (Ed.). pp 8-16. Schattauer Verlag, Stuttgart.

Gehmlich, K., C. Geier, K. J. Osterziel, P. F. M. Van der Ven & D. O. Fürst (2004). Decreased interactions of mutant muscle LIM protein (MLP) with N-RAP and alpha-actinin and their implication for hypertrophic cardiomyopathy. Cell Tiss. Res. 317, 129-136.

Kirfel, G., B. Borm, A. Rigort & V. Herzog, (2004), Cell Migration: Rear detachment and the formation of migration tracks. Eur. J. Cell Biol. 83, 717-724.

Kirfel, G. & V. Herzog (2004). Migration of epidermal keratinocytes: mechanisms, regulation and biological significance. Protoplasma 223, 67-78.

Maass, K., A. Ghanem, J. S. Kim, M. Saathoff, S. Urschel, G. Kirfel, R. Grümmer, M. Kretz, K. Tiemann, E. Winterhager, V. Herzog & K. Willecke (2004). Defective epidermal barrier in neonatal mice lacking the C-terminal region of connexin43. Mol. Biol. Cell. 15, 4597-4608.

Pacholsky, D., P. Vakeel, M. Himmel, T. Löwe, T. Stradal, K. Rottner, D. O. Fürst & P. F. M. van der Ven (2004). Xin repeats define a novel actin binding repeat. J. Cell Sci. 117, 5257-5268.

Rigort, A., J. Grünewald, V. Herzog & G. Kirfel (2004). Two types of integrin macroaggragates are released during rear detachment of migrating keratinocytes Eur. J. Cell Biol. 83, 725-733.

Saathoff, M., B. Blum, T. Quast, G. Kirfel & Herzog (2004). Simultaneous cell death and desquamation of the embryonic diffusion barrier during epidermal development. Exp. Cell Res. 209, 415-426.

2003

Andrade, F.H., A.P.Merriam, W.Guo, G.Cheng, C.A.McMullen, K.Hayess, P.F.M. van der Ven, & J.D.Porter. (2003). Paradoxical absence of M lines and downregulation of creatine kinase in mouse extraocular muscle. J Appl. Physiol 95, 692-699.

Bönnemann, C. G., T. G. Thompson, P. F. M. van der Ven, H. H. Goebel, J. Herms, M. Gautel, F. Takada, A. H. Beggs, D .O. Fürst, L.M. Kunkel, F. Hanefeld & R. Schröder (2003). Filamin C accumulation together with aB-crystallin is a strong but nonspecific immunohistochemical marker of core formation in muscle. J. Neurol. Sci. 206, 71-78.

Geier, C., A. Perrot, C. Özcelik, P. Binner, D. Counsell, K. Hoffmann, B. Pilz, Y. Martiniak, K. Gehmlich, P. F. M. van der Ven, D. O. Fürst, A. Vornwald, E. von Hodenberg, P. Nürnberg, T. Scheffold, R. Dietz & K. J. Osterziel (2003). Mutations in the human muscle LIM protein gene in families with hypertrophic cardiomyopathy. Circulation 107, 1390-1395.

Himmel, M., P. F. M. van der Ven, W. Stöcklein & D. O. Fürst (2003). The limits of promiscuity: isoform-specific dimerization of filamins. Biochemistry, 42, 430-439.

Hornemann, T., S. Kempa, M. Himmel, K. Hayess, D. O. Fürst & T. Wallimann (2003). Muscle type creatine kinase interacts with central domains of the M-band proteins myomesin and M

Kirfel, G., A. Rigort, B. Borm, C. Schulte & V. Herzog, V. (2003). Structural and compositional analysis of the keratinocyte migration track. Cell Motil. Cytoskeleton 55, 1-13.

Palenzuela, L., A.L.Andreu, J.Gamez, M.R.Vila, T.Kunimatsu, A.Meseguer, C.Cervera, C.Fernandez, I, P.F.M. van der Ven, T.G.Nygaard, E.Bonilla, & M.Hirano (2003). A novel autosomal dominant limb-girdle muscular dystrophy (LGMD 1F) maps to 7q32.1-32.2. Neurology 61, 404-406.

Petrides, P. E., D. O. Fürst & M. Gautel (2003). Muskelgewebe. in "Biochemie und Pathobiochemie", G. Löffler & K. Petrides (Ed.), 7. Auflage, Springer Verlag, Berlin.

Quast, T., S. Wehner, G. Kirfel & V. Herzog (2003). sAPP as a novel regulator of dendrite motility and melanin release in epidermal melanocytes and melanoma cells FASEB J. 17, 1739-41.

Salmikangas, P., P. F. M. van der Ven, A. Taivainen, F. Zhao, H. Suila, M. Lalowski, R. Schröder, P. Lappalainen, D. O. Fürst & O. Carpén (2003). Myotilin, an F-actin cross-linking protein, is required for proper assembly of the myofibril. Hum. Mol. Genet.12, 189-203.

Schröder, R. B. Goudeau, M. Casteras-Simon, D. Fischer, T. Eggermann, C. S. Clemen, Z. Li, J. Reimann, Z. Xue, A. A. Noegel, S. Rudnick-Schöneborn, K. Zerres, P. F. M. van der Ven, D. O. Fürst, W. S. Kunz & P. Vicart (2003). On noxious desmin: functional effects of a novel heterozygous desmin insertion mutation on the extrasarcomeric desmin cytoskeleton and mitochondria. Hum. Mol. Genet. 12, 657-669.

Yu, J.-G., D. O. Fürst & L.-E. Thornell (2003). The mode of myofibril remodeling in human skeletal muscle affected by DOMS induced by eccentric contractions. Histochem. Cell Biol. 119, 383-393.

ÄLTERE PUBLIKATIONEN / OLDER PUBLICATIONS

Schröder, R., W. S. Kunz, F. Rouan, E. Pfender, K. Tolksdorf, K. Kappes-Horn, M. Altenschmidt-Mehring, R. Knoblich, P. F. M. van der Ven, J. Reimann, D. O. Fürst, I. Blümcke, S. Vielhaber, D. Zillikens, S. Eming, T. Klockgether, J. Uitto, G. Wiche & A. Rolfs (2002). Disorganization of the desmin cytoskeleton and mitochondrial dysfunction in plectin-related muscular dystrophy with epidermolysis bullosa simplex. J. Neuropathol. Exp. Neurol. 61, 520-530.

Schröder , R., D. Pacholsky, J. Reimann, J. Matten, G. Wiche, D. O. Fürst & P. F. M. van der Ven (2002). Primary longitudinal adhesion structures (PLAS): plectin containing precursors of costameres in differentiating human skeletal muscle cells. Histochem. Cell Biol. 118, 301-310.

Pizon, V., A. Iakovenko, R. Kelly, P. F. M. van der Ven, D. O. Fürst, E. Karsenti & M. Gautel (2002). Transient association of titin and myosin with microtubules in nascent myofibrils directed by the MURF2 RING-finger protein. J. Cell Sci. 115, 4469-4482.

Sewry, C. A., C. Müller, M. Davis, J. S. M. Dwyer, J. Dove, G. Evans, R. Schröder, D. O. Fürst, T. Helliwell, N. Laing & R. C. M. Quinlivan (2002). The spectrum of pathology in central core disease. Neuromusc. Disorders 12, 930-938.

Kirfel, G., B. Borm, A. Rigort & V. Herzog (2002). The secretory beta-amyloid precursor protein is a motogen for human epidermal keratinocytes. Eur. J. Cell Biol. 81, 664-676.

Schmitz, A., R. Tikkanen, G. Kirfel & V. Herzog (2002). The biological role of the Alzheimer amyloid precursor protein in epithelial cells. Histochem. Cell Biol. 117, 171-180.

Guan, K., J. Czyz, D. O. Fürst & A. M. Wobus (2001). Expression and cellular distribution of

Van der Ven, P. F. M. 2001. Skeletal muscle. In Human Cell Culture Vol. V, Primary Mesenchymal Cells. M. R. Koller, B. O. Palsson, and J. R. W. Masters, editors. Kluwer Academic Publishers, Dordrecht. pp65-101

Chakharova , C., M. S. Wehnert, K. Uhl, S. Sakthivel, H.-P. Vosberg, P. F. M. van der Ven & D. O. Fürst (2000). Genomic structure and fine mapping of the two filamin gene paralogues FLNB and FLNC and comparative analysis of the filamin gene family. Hum. Genet. 107, 597-611.

Schröder, R., D. O.Fürst, C. Klasen, J. Reimann, H. Herrmann & P. F. M. van der Ven (2000). The association of plectin with Z-discs is a prerequisite for the formation of the intermyofibrillar desmin cytoskeleton. Lab. Invest. 80, 455-464.

Schröder, R., P. F. M. van der Ven, I. Warlo, H. Schumann, D. O. Fürst, I. Blümcke, H. H. Goebel, M. C. Schmidt & M. Hatzfeld (2000). p0071, a member of the armadillo multigene family, is a constituent of sarcomeric I-bands in human skeletal muscle. J. Musc. Res. Cell Motil. 21, 577-586.

Van der Ven, P. F. M., W. M. J. Obermann, B. Lemke, M. Gautel, K. Weber & D. O. Fürst (2000). The characterization of muscle filamin isoforms suggests a possible role of ABP-L/g-filamin in sarcomeric Z-disc formation. Cell Motil. Cytoskel. 45, 149-162.

Van der Ven, P. F.M., J. Bartsch, M. Gautel, H. Jockusch & D. O. Fürst (2000). A functional knock-out of titin results in defective myofibril assembly. J. Cell Sci. 113, 1405-1414.

Van der Ven, P. F. M., S. Wiesner, P. Salmikangas, D. Auerbach, M. Himmel, S. Kempa, K. Hayeß, D. Pacholsky, A. Taivainen, R. Schröder, O. Carpén & D. O. Fürst (2000). Indications for a novel muscular dystrophy pathway: g-filamin, the muscle-specific filamin isoform, interacts with myotilin. J. Cell Biol. 151, 235-248.

Fürst, D.O., R.A. Cross, J. DeMey & J.V. Small (1986). Caldesmon is an elongated, flexible molecule localized in the actomyosin domains of smooth muscle. EMBO J. 5, 251-257.

Small, J.V., D.O. Fürst & J. DeMey (1986). Localization of filamin in smooth muscle. J. Cell Biol. 102, 210-219.

Thornell, L.E., G.S. Butler-Browne, E. Carlsson, H.M. Eppenberger, D.O. Fürst, B.K. Grove, B. Holbom & J.V. Small (1986). Cryoultramicrotomy and immunocytochemistry in the analysis of muscle fine structure. Scanning EM IV, 1407-1418.

Fürst, D.O., R. Nave, M. Osborn, K. Weber, A. Bardosi, N. Archidiacono, M. Ferro, V. Romano & G. Romeo (1987). Nebulin and titin expression in Duchenne muscular dystrophy appears normal. FEBS Lett. 224, 49-53.

Gimona, M., D.O. Fürst & J.V. Small (1987). Metavinculin and vinculin from mammalian smooth muscle: bulk isolation and characterization. J. Musc. Res. Cell Mot. 8, 329-341.

Kossmann, T., D.O. Fürst & J.V. Small (1987). Structural and biochemical analysis of skinned smooth muscle preparations. J. Musc. Res. Cell Mot. 8, 135-144.

Fürst, D.O., M. Osborn, R. Nave & K. Weber (1988). The organization of titin filaments in the half-sarcomere revealed by monoclonal antibodies in immunoelectron microscopy; a map of 10 non repetitive epitopes starting at the Z-line extends close to the M-line. J.Cell Biol. 106, 1593 - 1605.

Fürst, D.O., M. Osborn & K. Weber (1989). Myogenesis in the mouse embryo: differential onset of expression of myogenic proteins and involvement of titin in myofibril assembly. J. Cell Biol. 109, 517 - 527.

Fürst, D.O., R. Nave, M. Osborn & K. Weber (1989). Repetitive titin epitopes with a 42 nm spacing coincide in relative position with known A band striations also identified by major myosin-associated proteins; an immunoelectron microscopical study on myofibrils. J. Cell Sci. 94, 119 - 125.

Nave, R., D.O. Fürst & K. Weber (1989). Visualization of the polarity of isolated titin molecules; a single globular head on a long thin rod as the M-band anchoring domain? J. Cell Biol. 109, 2177 - 2187.

Nave, R., D.O Fürst, & K. Weber (1990) Interaction of a-actinin and nebulin in vitro: support for the existence of a fourth filament system in skeletal muscle. FEBS Lett. 269, 163-166.

Joosten, E. A., P. F. M. van der Ven, M. H. Hooiveld, and H. J. ten Donkelaar. (1991). Induction of corticospinal target finding by release of a diffusible, chemotropic factor in cervical spinal grey matter. Neurosci. Lett. 128, 25-28.

Nave, R., D.O. Fürst, U. Vinkemeier & K. Weber ( 1991). Purification and physical properties of nematode mini-titin and their relation to twitchin. J. Cell Sci. 98, 491-496.

Van der Ven, P. F. M., P. H. K. Jap, R. H. Wetzels, H. J. ter Laak, F. C. S. Ramaekers, A. M. Stadhouders, and R. C. Sengers. (1991). Postnatal centralization of muscle fibre nuclei in centronuclear myopathy. Neuromuscul. Disord. 1:211-220.

Fürst, D.O., U. Vinkemeier & K. Weber (1992). Mammalian skeletal muscle C-protein: purification from bovine muscle, binding to titin and the characterization of a full-length human cDNA. J. Cell Sci. 102, 769-778.

Small, J.V., D.O. Fürst & L.-E. Thornell (1992). The cytoskeletal lattice of muscle cells (Invited Review). Eur. J. Biochem. 208, 559-572.

Schaart, G., P. F. M. van der Ven, and F. C. S.Ramaekers. (1993). Characterization of cardiotin, a structural component in the myocard. Eur. J Cell Biol. 62, 34-48.

Van Neck, J. W., J. J. Medina, C. Onnekink, P. F. M. van der Ven, H. P. Bloemers, & S.M.Schwartz. 1993. Basic fibroblast growth factor has a differential effect on MyoD conversion of cultured aortic smooth muscle cells from newborn and adult rats. Am. J Pathol. 143, 269-282.

Van der Ven, P. F. M., G. Schaart, P. H. K. Jap, R. C. Sengers, A. M. Stadhouders, & F. C. S. Ramaekers. (1992). Differentiation of human skeletal muscle cells in culture: maturation as indicated by titin and desmin striation. Cell Tissue Res. 270, 189-198.

Van der Ven, P. F. M., G. Schaart, H. J. Croes, P. H. K. Jap, L. A. Ginsel, & F. C. S. Ramaekers. (1993). Titin aggregates associated with intermediate filaments align along stress fiber-like structures during human skeletal muscle cell differentiation. J Cell Sci 106, 749-759.

Van der Ven, P. F. M., G. Jansen, T. H. van Kuppevelt, M. B. Perryman, M. Lupa, P. W. Dunne, H. J. ter Laak, P. H. K. Jap, J. H. Veerkamp, H. F. Epstein, and B. Wieringa. (1993). Myotonic dystrophy kinase is a component of neuromuscular junctions. Hum. Mol. Genet. 2, 1889-1894.

Vinkemeier, U., W. Obermann, K. Weber & D.O. Fürst (1993). The head domain of titin extends into the center of the sarcomeric M band; cDNA cloning, epitope mapping and immunoelectron microscopy of two titin associated proteins. J. Cell Sci. 106, 319-330.

Ausma, J., D. O. Fürst, F. Thone, B. Shivalkar, W. Flameng, K. Weber, F. Ramaekers & M. Borgers (1995). Molecular changes of titin in chronic hibernating myocardium as a characteristic of cardiomyocyte dedifferentiation. J. Mol. Cell. Cardiol. 27, 1203-1212.

Bender, R., M.A. Surani, R. Kothary, L.-L. Li, D.O. Fürst, B. Christ & R. Fundele (1995). Tissue specific loss of proliferative capacity of parthogenetic cells in fetal mouse chimeras. Roux´s Arch. Dev. Biol. 204, 436-443.

Fürst, D. O. & M. Gautel (1995). The anatomy of a molecular giant: how the sarcomere cytoskeleton is assembled from immunoglobulin superfamily molecules (Invited Review). J. Mol. Cell. Cardiol. 27, 951-959.

Obermann, W., U. Plessmann, K. Weber & D. O. Fürst (1995). Purification and biochemical characterization of myomesin, a myosin and titin binding protein, from bovine skeletal muscle. Eur. J. Biochem. 233, 110-115.

Van der Ven, P. F. M., P. H. K. Jap, P. G. Barth, R. C. Sengers, F. C. S.Ramaekers, & A. M. Stadhouders. 1995. Abnormal expression of intermediate filament proteins in X-linked myotubular myopathy is not reproduced in vitro. Neuromuscul. Disord. 5:267-275.

Van der Ven, P. F. M., P. H. K. Jap, H. J. ter Laak, I. Nonaka, P. G. Barth, R. C. Sengers, A. M. Stadhouders, & F. C. S. Ramaekers. 1995. Immunophenotyping of congenital myopathies: disorganization of sarcomeric, cytoskeletal and extracellular matrix proteins. J Neurol. Sci 129, 199-213.

Van der Ven, P. F. M., A. Y. Wei, P. H. K. Jap, H. A. van der Lee, F. M. Debruyne, and E. J. Meuleman. (1995). Increased expression of a 68-kDa protein in the corpus cavernosum of some men with erectile dysfunction. J Androl 16, 242-247.

Gautel, M., D. Goulding, B. Bullard, K. Weber & D. O. Fürst (1996). The central Z-disk region of titin is assembled from a novel repeat in variable copy numbers. J. Cell Sci. 109, 2747-2754

Obermann, W., M. Gautel, F. Steiner, P.F.M. van der Ven, K. Weber & D.O. Fürst (1996). Molecular structure of the sarcomeric M band: localization of defined domains of myomesin, M-protein and the 250 kDa carboxyterminal region of titin. J. Cell Biol., 134, 1441-1453.

Van der Loop, F. T. L., P. F. M. van der Ven, D. O. Fürst, M. Gautel, G. J. J. M. van Eys & F. C. S. Ramaekers (1996). Integration of titin into the sarcomeres of cultured differentiating human skeletal muscle cells. Eur. J. Cell Biol. 69, 301-307.

Van der Ven, P.F.M., W. Obermann, K. Weber & D. O. Fürst (1996). Myomesin, M-protein and the structure of the sarcomeric M-band. Adv. Biophys. 33, 91-99.

Giebing, T., W. Obermann, D. O. Fürst & J. D´Haese (1997). C-terminally deleted fragments of 40-kDa earthworm actin modulator still shows gelsolin activities. FEBS Lett. 417, 191-195.

Obermann, W., M. Gautel, K. Weber & D. O. Fürst (1997). Molecular structure of the sarcomeric M band: identification of a novel PKA phosphorylation site and mapping of titin- and myosin-binding domains in myomesin. EMBO J., 16, 211-220.

Van der Ven, P. F. M. & D. O. Fürst (1997). Assembly of titin, myomesin and M-protein into the sarcomeric M band in differentiating human skeletal muscle cells in vitro. Cell Struct. Funct. 22, 163-171.

Gautel, M., D. O. Fürst, A. Cocco & S. Schiaffino (1998). Isoform transitions of the myosin- binding protein C family in developing human and mouse muscles - lack of isoform transcomplementation in cardiac muscle. Circ. Res. 82, 124-129.

Mayans, O., P. F. M. van der Ven, M. Wilm, A. Mues, P. Young, D. O. Fürst, M. Wilmanns & M. Gautel (1998). The structural basis of the activation of the serine kinase domain of the giant muscle protein titin during myofibrillogenesis. Nature 395, 863-869.

Mues, A., P. F. M. van der Ven, P. Young, D. O. Fürst & M. Gautel (1998). Two immunoglobulin-like domains of the Z-disk portion of titin interact in a conformation-dependent way with telethonin. FEBS Lett. 428, 111-114.

Obermann, W., P. F. M. van der Ven, F. Steiner, K. Weber & D. O. Fürst (1998). Mapping of a myosin binding domain and a regulatory phosphorylation site in M-protein, a structural protein of the sarcomeric M band. Mol. Biol. Cell 9, 829-840.

Rohwedel, J., K. Guan, W. Zuschratter, G. Ahnert-Hilger, D. Fürst, R. Fässler & A. M. Wobus (1998). Loss of b1 integrin function influences the differentiation of embryonic stem cells into the myogenic and neurogenic lineage. Dev. Biol. 201, 167-184.

Speel E. J. M., P. F. M. van der Ven, J. C. M. Albrechts, F. C. S. Ramaekers, D. O. Fürst & A. H. N. Hopman (1998). Chromosomal assignment of the human myomesin gene. Genomics 54, 184-186.

Steiner, F., K. Weber & D. O. Fürst (1998). Structure and expression of the gene encoding murine M-protein, a sarcomere-specific member of the immunoglobulin superfamily. Genomics 49, 83-95.

Van der Ven, P. F. M. & D. O. Fürst (1998). Expression of sarcomeric proteins and assembly of myofibrils in the putative myofibroblast cell line BHK-21/C13. J. Musc. Res. Cell Motil. 19, 767-775.

Bennett, P. M., D. O. Fürst & M. Gautel (1999). The C-protein (myosin binding protein C) family: regulators of contraction and sarcomere function? (Invited Review) Rev. Physiol. Biochem. Pharmacol. 138, 203-234.

Fürst, D. O. (1999). Titin, ein molekularer Gigant regiert im quergestreiften Muskel. Dt. Z. Sportmed. 50, 218-222.

Fürst, D. O., W. Obermann & P. F. M. van der Ven (1999). Structure and assembly of the sarcomeric M band (Invited Review). Rev. Physiol. Biochem. Pharmacol. 138, 163-202.

Guan, K., D. O. Fürst & A. M. Wobus (1999). Modulation of sarcomere assembly during embryonic stem cell-derived cardiomyocyte differentiation. Eur. J. Cell Biol. 78, 813-823.

Perriard, J.-P. & D. O. Fürst (1999). Myomesin and M-Protein. in: Guidebook to Cytoskeletal and Motor Proteins; T. Kreis & R. Vale (Eds.), pp. 475-478, Oxford University Press, Oxford.

Pette, D. & D. O. Fürst, Eds. (1999). The Third Filament System. Rev. Physiol. Biochem. Pharmacol. 138. Springer Verlag, Heidelberg.

Schröder, R., I. Warlo, H. Herrmann, P. F. M. van der Ven, C. Klasen, I. Blümcke, R. M. Mundegar, D. O. Fürst, H. H. Göbel & T. Magin (1999). Immunogold EM reveals a close association of plectin and the desmin cytoskeleton in human skeletal muscle. Eur. J. Cell Biol. 78, 288-295.

Steiner, F., K. Weber & D. O. Fürst (1999). M-band proteins myomesin and skelemin are encoded by a single gene: analysis of its organization and expression. Genomics 56, 78-89.

Van der Ven, P. F. M., E. J. M. Speel, J. C. M. Albrechts, F. C. S. Ramaekers, A. H. N. Hopman & D. O. Fürst (1999). Chromosomal assignment of the human gene for endosarcomeric cytoskeletal M-protein (MYOM2) to 8p23.3. Genomics 55, 253-255.

Van der Ven, P. F. M., E. Ehler, J.-C. Perriard & D. O. Fürst (1999). Thick filament assembly occurs after the formation of a cytoskeletal scaffold. J. Musc. Res. Cell Motil. 20, 569-579.
 
 
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