@article{VorgerdvanderVenBruchertseiferetal.2005, author = {Vorgerd, M. and vanderVen, Peter F. M. and Bruchertseifer, V. and Lowe, T. and Kley, R. A. and Schr{\"o}der, Rolf and Lochmuller, H. and Himmel, Mirko and Koehler, K. and F{\"u}rst, Dieter Oswald and Huebner, A.}, title = {A mutation in the dimerization domain of filamin C causes a novel type of autosomal dominant myofibrillar myopathy}, issn = {0002-9297}, year = {2005}, abstract = {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}, language = {en} } @article{UhlemannKuemmelSchroederetal.1996, author = {Uhlemann, Erhard and K{\"u}mmel, Rolf and Schr{\"o}der, Martina and Mickler, Wulfhard}, title = {Selektiver Ionentransport durch fl{\"u}ssige Membranen}, year = {1996}, language = {de} } @article{MeinckeRaumSchroederetal.1995, author = {Meincke, Rolf and Raum, Bernd and Schr{\"o}der, Ulrich and Uhlenwinkel, Anke}, title = {Terra Erdkunde : Arbeitsheft f{\"u}r Baden-W{\"u}rttemberg 5 / 6}, publisher = {Perthes}, address = {Gotha}, pages = {34 S.}, year = {1995}, language = {de} } @article{EhrlichmannKrumreinKugleretal.1996, author = {Ehrlichmann, Frank and Krumrein, Frieder and Kugler, Brigitte and Meincke, Rolf and Raum, Bernd and Schr{\"o}der, Ulrich and Uhlenwinkel, Anke and Zimmermann, Bernd and Zoska, Marianne}, title = {Terra Erdkunde : Arbeitsheft f{\"u}r Berlin 5 / 6 ; Lehrerheft}, publisher = {Perthes}, address = {Gotha}, pages = {32 S.}, year = {1996}, language = {de} } @article{EhrlichmannKrumreinKugleretal.1995, author = {Ehrlichmann, Frank and Krumrein, Frieder and Kugler, Brigitte and Meincke, Rolf and Raum, Bernd and Schr{\"o}der, Ulrich and Uhlenwinkel, Anke and Zimmermann, Bernd}, title = {Terra Erdkunde : Arbeitsheft f{\"u}r Berlin 5 / 6}, publisher = {Perthes}, address = {Gotha}, pages = {34 S.}, year = {1995}, language = {de} } @article{EhrlichmannKrumreinMeinckeetal.1995, author = {Ehrlichmann, Frank and Krumrein, Frieder and Meincke, Rolf and Raum, Bernd and Schr{\"o}der, Ulrich and Uhlenwinkel, Anke}, title = {Terra Erdkunde : Arbeitsheft f{\"u}r Brandenburg ; Lehrerheft}, publisher = {Perthes}, address = {Gotha}, pages = {36 S.}, year = {1995}, language = {de} } @article{SchroederKuemmelMickleretal.1991, author = {Schr{\"o}der, Martina and K{\"u}mmel, Rolf and Mickler, Wulfhard and Uhlemann, Erhard}, title = {Zur Abtrennung von Kupfer aus ammoniakalischer L{\"o}sung durch Fl{\"u}ssig-Fl{\"u}ssig-Extraktion und Fl{\"u}ssigmembranpermeation mit 1-Phenyl-3-methyl-4-stearoylpyrazol-5-on}, year = {1991}, language = {de} } @article{WiesnerSalmikangasAuerbachetal.2000, author = {Wiesner, Sebastian and Salmikangas, Paula and Auerbach, Daniel and Himmel, Mirko and Kempa, Stefan and Hayes, Kathrin and Pacholsky, Dirk and Taivainen, Anu and Schr{\"o}der, Rolf and Carpen, Olli and F{\"u}rst, Dieter Oswald}, title = {Indications for a novel muscular dystrophy pathway : gamma-filamin, the muscle-specific filamin isoform, intgeracts with myotilin}, year = {2000}, language = {en} } @article{SchroederVanDerVenWarloetal.2000, author = {Schr{\"o}der, Rolf and VanDerVen, Peter F. M. and Warlo, Irene and Schumann, H. and F{\"u}rst, Dieter Oswald and Bl{\"u}mke, Ingmar and Goebel, Hans H. and Schmidt, M. C. and Hatzfeld, Mechthild}, title = {A member of the armadillo multigene family, is a constituent of sarcomeric I-bands in human skeletal muscle}, year = {2000}, language = {en} } @article{SchroederFuerstKlasenetal.2000, author = {Schr{\"o}der, Rolf and F{\"u}rst, Dieter Oswald and Klasen, Christian and Reiman, Jens and Herrmann, Harald and VanDerVen, Peter F. M.}, title = {The association of plectin with Z-discs is a prerequisite for the formation of the intermyofibrillar desmin cytoskeleton}, year = {2000}, language = {en} } @article{SchroederWarloHerrmannetal.1999, author = {Schr{\"o}der, Rolf and Warlo, Irene and Herrmann, Harald and VanDerVen, Peter F. M and Klasen, Christian and Bl{\"u}mke, Ingmar and Mundegar, Rustam R. and F{\"u}rst, Dieter Oswald and G{\"o}bel, Hans H. and Magin, Thomas}, title = {Immunogold EM reveals a close association of plectin and the desmin cytoskeleton in human skeletal muscle}, year = {1999}, language = {en} } @article{MitznerRehanekKernetal.2013, author = {Mitzner, Rolf and Rehanek, Jens and Kern, Jan and Gul, Sheraz and Hattne, Johan and Taguchi, Taketo and Alonso-Mori, Roberto and Tran, Rosalie and Weniger, Christian and Schr{\"o}der, Henning and Quevedo, Wilson and Laksmono, Hartawan and Sierra, Raymond G. and Han, Guangye and Lassalle-Kaiser, Benedikt and Koroidov, Sergey and Kubicek, Katharina and Schreck, Simon and Kunnus, Kristjan and Brzhezinskaya, Maria and Firsov, Alexander and Minitti, Michael P. and Turner, Joshua J. and M{\"o}ller, Stefan and Sauter, Nicholas K. and Bogan, Michael J. and Nordlund, Dennis and Schlotter, William F. and Messinger, Johannes and Borovik, Andrew S. and Techert, Simone and de Groot, Frank M. F. and F{\"o}hlisch, Alexander and Erko, Alexei and Bergmann, Uwe and Yachandra, Vittal K. and Wernet, Philippe and Yano, Junko}, title = {L-edge x-ray absorption spectroscopy of dilute systems relevant to metalloproteins using an X-ray free-electron laser}, series = {The journal of physical chemistry letters}, volume = {4}, journal = {The journal of physical chemistry letters}, number = {21}, publisher = {American Chemical Society}, address = {Washington}, issn = {1948-7185}, doi = {10.1021/jz401837f}, pages = {3641 -- 3647}, year = {2013}, abstract = {L-edge spectroscopy of 3d transition metals provides important electronic structure information and has been used in many fields. However, the use of this method for studying dilute aqueous systems, such as metalloenzymes, has not been prevalent because of severe radiation damage and the lack of suitable detection systems. Here we present spectra from a dilute Mn aqueous solution using a high-transmission zone-plate spectrometer at the Linac Coherent Light Source (LCLS). The spectrometer has been optimized for discriminating the Mn L-edge signal from the overwhelming 0 K-edge background that arises from water and protein itself, and the ultrashort LCLS X-ray pulses can outrun X-ray induced damage. We show that the deviations of the partial-fluorescence yield-detected spectra from the true absorption can be well modeled using the state-dependence of the fluorescence yield, and discuss implications for the application of our concept to biological samples.}, language = {en} }