Zobrazeno 1 - 10
of 45
pro vyhledávání: 'Lüllmann-Rauch, Renate'
Autor:
Rudi D'Hooge, Cecilia Weigelt, Volkmar Gieselmann, Claes Andersson, Carl Eistrup, Ulrich Matzner, Jens Fogh, Renate Lüllmann-Rauch, Stijn Stroobants
Publikováno v:
Molecular Therapy. 17(4):600-606
Inherited deficiencies of lysosomal hydrolases cause lysosomal storage diseases (LSDs) that are characterized by a progressive multisystemic pathology and premature death. Repeated intravenous injection of the active counterpart of the deficient enzy
Autor:
Hans Bockhardt, Renate Lüllmann-Rauch
Publikováno v:
Acta Pharmacologica et Toxicologica. 47:45-48
This study was aimed at the question of whether or not the antidepressant zimelidine, which is an amphiphilic cationic compound, can induce generalized lipidosis in animals. Rats were chronically treated with high oral doses (80 mg/kg) of the drug, a
Autor:
Stijn Stroobants, Paul Saftig, Renate Lüllmann-Rauch, Claes Andersson, Rudi D'Hooge, Meike Lüdemann, Jens Fogh, Willy Morelle, Judith Blanz, Helena Reuterwall, Jean Claude Michalski
Publikováno v:
Human Molecular Genetics. 17:3437-3445
Despite the progress in the treatment of lysosomal storage disorders (LSDs) mainly by enzyme replacement therapy, only limited success was reported in targeting the appropriate lysosomal enzyme into the brain. This prevents efficient clearance of neu
Autor:
Volkmar Gieselmann, Kerstin Khalaj Hedayati, Hariharasubramanian Ramakrishnan, Hans-Hilmar Goebel, Renate Lüllmann-Rauch, Simon Ngamli Fewou, Matthias Eckhardt, Carsten Wessig, Helena Maier
Publikováno v:
The Journal of Neuroscience. 27:9482-9490
Metachromatic leukodystrophy (MLD) is a lysosomal storage disorder caused by the deficiency of arylsulfatase A (ASA). This results in accumulation of sulfated glycosphingolipids, mainly 3-O-sulfogalactosylceramide (sulfatide), in the nervous system a
Autor:
Ulrike Zimmermann, Eeva-Liisa Eskelinen, Paul Saftig, Michael Schwake, Renate Lüllmann-Rauch, Lukas Rüttiger, Cathrin Claussen, Jenny Schröder, Marlies Knipper
Publikováno v:
The Journal of Physiology. 576:73-86
Our previous studies revealed a critical role of the lysosomal membrane protein LIMP2 in the regulation of membrane transport processes in the endocytic pathway. Here we show that LIMP2-deficient mice display a progressive high-frequency hearing loss
Autor:
P. Fredman, Ulrich Matzner, Rudi D'Hooge, P.P. De Deyn, Dieter Hartmann, Renate Lüllmann-Rauch, F. Rothert, Volkmar Gieselmann, Jan-Eric Månsson, R. Coenen
Publikováno v:
Gene therapy
Arylsulfatase A (ASA) knockout mice represent an animal model for the lysosomal storage disease metachromatic leukodystrophy (MLD). Stem cell gene therapy with bone marrow overexpressing the human ASA cDNA from a retroviral vector resulted in the exp
Autor:
Sebastian Franken, Volkmar Gieselmann, Ulrich Matzner, Renate Lüllmann-Rauch, Dieter Hartmann
Publikováno v:
Histochemistry and Cell Biology. 116:161-169
The inherited deficiency of arylsulfatase A (ASA) causes lysosomal accumulation of sulfoglycolipids (mainly sulfo-galactosylceramide, S-GalCer ) and leads to metachromatic leukodystrophy in humans. Among visceral organs, kidneys are particularly affe
Publikováno v:
Acta Neuropathologica. 101:491-498
Metachromatic leukodystrophy of humans is an inherited sulfatide lipidosis due to deficiency of arylsulfatase A (ASA). As an animal model, ASA(-/-) mice have been generated. A previous study showed that the mice lose most of their spiral (acoustic) g
Publikováno v:
Virchows Archiv. 439:90-96
The inherited deficiency of arylsulfatase A (ASA) in humans causes lysosomal accumulation of sulfatides in visceral organs and in the nervous system and leads to wide-spread demyelination (metachromatic leukodystrophy, MLD). ASA-deficient mice have p