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  - Uhlemann, Erhard
A1  - Kümmel, Rolf
A1  - Schröder, Martina
A1  - Mickler, Wulfhard
T1  - Selektiver Ionentransport durch flüssige Membranen
Y1  - 1996
ER  - 
TY  - JOUR
A1  - Meincke, Rolf
A1  - Raum, Bernd
A1  - Schröder, Ulrich
A1  - Uhlenwinkel, Anke
T1  - Terra Erdkunde : Arbeitsheft für Baden-Württemberg 5 / 6
Y1  - 1995
PB  - Perthes
CY  - Gotha
ER  - 
TY  - JOUR
A1  - Ehrlichmann, Frank
A1  - Krumrein, Frieder
A1  - Kugler, Brigitte
A1  - Meincke, Rolf
A1  - Raum, Bernd
A1  - Schröder, Ulrich
A1  - Uhlenwinkel, Anke
A1  - Zimmermann, Bernd
A1  - Zoska, Marianne
T1  - Terra Erdkunde : Arbeitsheft für Berlin 5 / 6 ; Lehrerheft
Y1  - 1996
PB  - Perthes
CY  - Gotha
ER  - 
TY  - JOUR
A1  - Ehrlichmann, Frank
A1  - Krumrein, Frieder
A1  - Kugler, Brigitte
A1  - Meincke, Rolf
A1  - Raum, Bernd
A1  - Schröder, Ulrich
A1  - Uhlenwinkel, Anke
A1  - Zimmermann, Bernd
T1  - Terra Erdkunde : Arbeitsheft für Berlin 5 / 6
Y1  - 1995
PB  - Perthes
CY  - Gotha
ER  - 
TY  - JOUR
A1  - Ehrlichmann, Frank
A1  - Krumrein, Frieder
A1  - Meincke, Rolf
A1  - Raum, Bernd
A1  - Schröder, Ulrich
A1  - Uhlenwinkel, Anke
T1  - Terra Erdkunde : Arbeitsheft für Brandenburg ; Lehrerheft
Y1  - 1995
PB  - Perthes
CY  - Gotha
ER  - 
TY  - JOUR
A1  - Schröder, Martina
A1  - Kümmel, Rolf
A1  - Mickler, Wulfhard
A1  - Uhlemann, Erhard
T1  - Zur Abtrennung von Kupfer aus ammoniakalischer Lösung durch Flüssig-Flüssig-Extraktion und Flüssigmembranpermeation mit 1-Phenyl-3-methyl-4-stearoylpyrazol-5-on
Y1  - 1991
ER  - 
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  - 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  - 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  - Mitzner, Rolf
A1  - Rehanek, Jens
A1  - Kern, Jan
A1  - Gul, Sheraz
A1  - Hattne, Johan
A1  - Taguchi, Taketo
A1  - Alonso-Mori, Roberto
A1  - Tran, Rosalie
A1  - Weniger, Christian
A1  - Schröder, Henning
A1  - Quevedo, Wilson
A1  - Laksmono, Hartawan
A1  - Sierra, Raymond G.
A1  - Han, Guangye
A1  - Lassalle-Kaiser, Benedikt
A1  - Koroidov, Sergey
A1  - Kubicek, Katharina
A1  - Schreck, Simon
A1  - Kunnus, Kristjan
A1  - Brzhezinskaya, Maria
A1  - Firsov, Alexander
A1  - Minitti, Michael P.
A1  - Turner, Joshua J.
A1  - Möller, Stefan
A1  - Sauter, Nicholas K.
A1  - Bogan, Michael J.
A1  - Nordlund, Dennis
A1  - Schlotter, William F.
A1  - Messinger, Johannes
A1  - Borovik, Andrew S.
A1  - Techert, Simone
A1  - de Groot, Frank M. F.
A1  - Föhlisch, Alexander
A1  - Erko, Alexei
A1  - Bergmann, Uwe
A1  - Yachandra, Vittal K.
A1  - Wernet, Philippe
A1  - Yano, Junko
T1  - L-edge x-ray absorption spectroscopy of dilute systems relevant to metalloproteins using an X-ray free-electron laser
JF  - The journal of physical chemistry letters
N2  - 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.
Y1  - 2013
U6  - https://doi.org/10.1021/jz401837f
SN  - 1948-7185
VL  - 4
IS  - 21
SP  - 3641
EP  - 3647
PB  - American Chemical Society
CY  - Washington
ER  -