TY  - JOUR
A1  - Wiesner, Sebastian
A1  - Salmikangas, Paula
A1  - Auerbach, Daniel
A1  - Himmel, Mirko
A1  - Kempa, Stefan
A1  - Hayes, Kathrin
A1  - Pacholsky, Dirk
A1  - Taivainen, Anu
A1  - Schröder, Rolf
A1  - Carpen, Olli
A1  - Fürst, Dieter Oswald
T1  - Indications for a novel muscular dystrophy pathway : gamma-filamin, the muscle-specific filamin isoform, intgeracts with myotilin
Y1  - 2000
ER  - 
TY  - JOUR
A1  - Vorgerd, M.
A1  - vanderVen, Peter F. M.
A1  - Bruchertseifer, V.
A1  - Lowe, T.
A1  - Kley, R. A.
A1  - Schröder, Rolf
A1  - Lochmuller, H.
A1  - Himmel, Mirko
A1  - Koehler, K.
A1  - Fürst, Dieter Oswald
A1  - Huebner, A.
T1  - A mutation in the dimerization domain of filamin C causes a novel type of autosomal dominant myofibrillar myopathy
N2  - Myofibrillar myopathy (MFM) is a human disease that is characterized by focal myofibrillar destruction and pathological cytoplasmic protein aggregations. In an extended German pedigree with a novel form of MFM characterized by clinical features of a limb-girdle myopathy and morphological features of MFM, we identified a cosegregating, heterozygous nonsense mutation (8130G -> A; W2710X) in the filamin c gene ( FLNC) on chromosome 7q32.1. The mutation is the first found in FLNC and is localized in the dimerization domain of filamin c. Functional studies showed that, in the truncated mutant protein, this domain has a disturbed secondary structure that leads to the inability to dimerize properly. As a consequence of this malfunction, the muscle fibers of our patients display massive cytoplasmic aggregates containing filamin c and several Z-disk-associated and sarcolemmal proteins
Y1  - 2005
SN  - 0002-9297
ER  - 
TY  - JOUR
A1  - VanDerVen, Peter F. M.
A1  - Speel, Ernst J. M.
A1  - Albrechts, Jozefa C. M.
A1  - Ramaekers, Frans C. S.
A1  - Hopman, Anton H. N.
A1  - Fürst, Dieter Oswald
T1  - Chromosomal assignment of the human gene for endosarcomeric cytoskeletal M-protein (MYOM2) to 8p23.3
Y1  - 1999
ER  - 
TY  - JOUR
A1  - VanDerVen, Peter F. M.
A1  - Speel, Ernst J. M.
A1  - Albrechts, Jozefa C. M.
A1  - Ramaekers, Frans C. S.
A1  - Hopman, Anton H. N.
A1  - Fürst, Dieter Oswald
T1  - Assignment of the human gene for endosarcomeric cytoskeletal M-protein (MYOM2) to 8p23.3
Y1  - 1999
ER  - 
TY  - JOUR
A1  - VanderVen, Peter F. M.
A1  - Ehler, Elisabeth
A1  - Vakeel, Padmanabhan
A1  - Eulitz, Stefan
A1  - Schenk, Jörg A.
A1  - Milting, Hendrik
A1  - Micheel, Burkhard
A1  - Fürst, Dieter Oswald
T1  - Unusual splicing events result in distinct Xin isoforms that associate differentially with filamin c and Mena/ VASP
N2  - Filamin c is the predominantly expressed filamin isoform in striated muscles. It is localized in myofibrillar Z- discs, where it binds FATZ and myotilin, and in myotendinous junctions and intercalated discs. Here, we identify Xin, the protein encoded by the human gene 'cardiomyopathy associated 1' (CMYA1) as filamin c binding partner at these specialized structures where the ends of myofibrils are attached to the sarcolemma. Xin directly binds the EVH1 domain proteins Mena and VASP. In the adult heart, Xin and Mena/VASP colocalize with filamin c in intercalated discs. In cultured cardiomyocytes, the proteins also localize in the nonstriated part of myofibrils, where sarcomeres are assembled and an extensive reorganization of the actin cytoskeleton occurs. Unusual intraexonic splicing events result in the existence of three Xin isoforms that associate differentially with its ligands. The identification of the complex filamin c-Xin-Mena/VASP provides a first glance on the role of Xin in the molecular mechanisms involved in developmental and adaptive remodeling of the actin cytoskeleton during cardiac morphogenesis and sarcomere assembly. (c) 2006 Elsevier Inc. All rights reserved
Y1  - 2006
U6  - https://doi.org/10.1016/j.yexcr.2006.03.015
ER  - 
TY  - JOUR
A1  - VanDerVen, Peter F. M.
A1  - Bartsch, Jörg
A1  - Gautel, Mathias
A1  - Jokusch, Harald
A1  - Fürst, Dieter Oswald
T1  - A functional knock-out of titin results in defective myofibril assembly
Y1  - 2000
ER  - 
TY  - JOUR
A1  - VanDerVen, Peter F. M
A1  - Obermann, Wolfgang
A1  - Weber, Klaus
A1  - Fürst, Dieter Oswald
T1  - Myomesin, M-protein and the structure of the sarcomeric M-band
Y1  - 1996
ER  - 
TY  - JOUR
A1  - VanDerVen, Peter F. M
A1  - Obermann, Wolfgang
A1  - Lemke, Britt
A1  - Gautel, Mathias
A1  - Weber, Klaus
A1  - Fürst, Dieter Oswald
T1  - The characterization of muscle filamin isoforms suggests a possible role of ABP-L/gamma-filamin in sarcomeric Z- disc formation
Y1  - 2000
ER  - 
TY  - JOUR
A1  - VanDerVen, Peter F. M
A1  - Fürst, Dieter Oswald
T1  - Expression of sarcomeric proteins and assembly of myofibrils in the putative myofibroblast cell line BHK-21/C13
Y1  - 1998
ER  - 
TY  - JOUR
A1  - VanDerVen, Peter F. M
A1  - Fürst, Dieter Oswald
T1  - Assembly of titin, myomesin and M-protein into the sarcomeric M-band in differentiating human skeletal muscle cells in vitro
Y1  - 1997
ER  - 
TY  - JOUR
A1  - VanDerVen, Peter
A1  - Ehler, Elisabeth
A1  - Perriard, Jean-Claude
A1  - Fürst, Dieter Oswald
T1  - Thick filament assembly occurs after the formation of a cytoskeletal scaffold.
Y1  - 1999
ER  - 
TY  - JOUR
A1  - VanDerLoop, Frank T. L.
A1  - VanDerVen, Peter F. M
A1  - Fürst, Dieter Oswald
A1  - Gautel, Mathias
A1  - VanEys, Guillaume
A1  - Ramaekers, Frans C. S.
T1  - Integration of titin into the sarcomeres of cultured differentiating human skeletal muscle cells
Y1  - 1996
ER  - 
TY  - JOUR
A1  - Steiner, F.
A1  - Weber, Klaus
A1  - Fürst, Dieter Oswald
T1  - Structure and expression of the gene encoding murine M-protein, a sarcomere-specific member of the immunoglobulin superfamily
Y1  - 1998
ER  - 
TY  - JOUR
A1  - Steiner, F.
A1  - Fürst, Dieter Oswald
A1  - Weber, Klaus
T1  - M-band proteins myomesin und skelemin are encoded by a single gene: analysis of its organization and expression
Y1  - 1999
ER  - 
TY  - JOUR
A1  - Speel, Ernst J. M.
A1  - VanDerVen, Peter F. M.
A1  - Albrechts, Jozefa C. M.
A1  - Ramaekers, Frans C. S.
A1  - Fürst, Dieter Oswald
A1  - Hopman, Anton H. N.
T1  - Assignment of the human gene for the sarcomeric M-band protein myomesin (MYOM1) to 18p11.31-p11.32
Y1  - 1998
ER  - 
TY  - JOUR
A1  - Speel, Ernst J. M.
A1  - VanDerVen, Peter F. M.
A1  - Albrechts, Jozefa C. M.
A1  - Hopman, Anton H. N.
A1  - Fürst, Dieter Oswald
T1  - Chromosomal assignment of the human myomesin gene
Y1  - 1998
ER  - 
TY  - JOUR
A1  - Schröder, Rolf
A1  - Warlo, Irene
A1  - Herrmann, Harald
A1  - VanDerVen, Peter F. M
A1  - Klasen, Christian
A1  - Blümke, Ingmar
A1  - Mundegar, Rustam R.
A1  - Fürst, Dieter Oswald
A1  - Göbel, Hans H.
A1  - Magin, Thomas
T1  - Immunogold EM reveals a close association of plectin and the desmin cytoskeleton in human skeletal muscle
Y1  - 1999
ER  - 
TY  - JOUR
A1  - Schröder, Rolf
A1  - VanDerVen, Peter F. M.
A1  - Warlo, Irene
A1  - Schumann, H.
A1  - Fürst, Dieter Oswald
A1  - Blümke, Ingmar
A1  - Goebel, Hans H.
A1  - Schmidt, M. C.
A1  - Hatzfeld, Mechthild
T1  - A member of the armadillo multigene family, is a constituent of sarcomeric I-bands in human skeletal muscle
Y1  - 2000
ER  - 
TY  - JOUR
A1  - Schröder, Rolf
A1  - Fürst, Dieter Oswald
A1  - Klasen, Christian
A1  - Reiman, Jens
A1  - Herrmann, Harald
A1  - VanDerVen, Peter F. M.
T1  - The association of plectin with Z-discs is a prerequisite for the formation of the intermyofibrillar desmin cytoskeleton
Y1  - 2000
ER  - 
TY  - JOUR
A1  - Rohwedel, Jürgen
A1  - Guan, Kaomei
A1  - Zuschratter, W.
A1  - Ahnert-Hilger, Shan Jin Gudrun
A1  - Fürst, Dieter Oswald
A1  - Fässler, Reinhard
A1  - Wobus, Anna
T1  - Loss of beta 1 integrin function influences the differentiation of embryonic stem cells into the myogenic and neurogenic lineage
Y1  - 1998
ER  -