Zobrazeno 91 - 100
of 495
pro vyhledávání: 'Lüllmann-Rauch, Renate'
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:
Jérémie Gautheron, Stefan Rose-John, Matthias Lutz, Christoph Jacoby, Florian Reisinger, Hans-Joerg Hippe, Dieter Adam, Christoph Roderburg, Andreas Linkermann, Mihael Vucur, Justyna Sosna, Mark Luedde, Nadine Borg, Ulrich Flögel, Monika Julia Wolf, Natalie Carter, Mathias Heikenwalder, Tom Luedde, Norbert Frey, Renate Lüllmann-Rauch
Aims Programmed necrosis (necroptosis) represents a newly identified mechanism of cell death combining features of both apoptosis and necrosis. Like apoptosis, necroptosis is tightly regulated by distinct signalling pathways. A key regulatory role in
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::3625612ef9e64d8333f3b5ccf6660593
https://doi.org/10.5167/uzh-154187
https://doi.org/10.5167/uzh-154187
Autor:
Bernd Schröder, Susann Hüttl, Jürgen Wienands, Kai Dittmann, Ralf Dressel, Michelle Rothaug, Paul Saftig, Michael Engelke, Regina Fluhrer, Torben Mentrup, Janna Schneppenheim, Masatake Araki, Kimi Araki, Renate Lüllmann-Rauch
Publikováno v:
Molecular and cellular biology 34(8), 1398-1411 (2014). doi:10.1128/MCB.00038-14
We reported recently that the presenilin homologue signal peptide peptidase-like 2a (SPPL2a) is essential for B cell development by cleaving the N-terminal fragment (NTF) of the invariant chain (li, CD74). Based on this, we suggested that pharmacolog
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::290f81def68c61c187030a7631e89458
Publikováno v:
Graefe's Archive for Clinical and Experimental Ophthalmology. 239:788-793
A series of dicationic amphiphilic drugs, most of them immunomodulatory agents, are known to induce generalised lysosomal storage of sulphated glycosaminoglycans (GAGs) in rats and in cultured cells of several species including man. The present study
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
Autor:
Renate Lüllmann-Rauch, Dieter Hartmann, Paul Saftig, Eeva Punnonen, Kurt von Figura, Gundula Guhde, Nicole Andrejewski, Yoshitaka Tanaka
Publikováno v:
Journal of Biological Chemistry. 274:12692-12701
Lysosomal membranes contain two highly glycosylated proteins, designated LAMP-1 and LAMP-2, as major components. LAMP-1 and LAMP-2 are structurally related. To investigate the physiological role of LAMP-1, we have generated mice deficient for this pr
Autor:
Damme, Markus, Stroobants, Stijn, Lüdemann, Meike, Rothaug, Michelle, Lüllmann‐Rauch, Renate, Beck, Hans Christian, Ericsson, Annika, Andersson, Claes, Fogh, Jens, D'Hooge, Rudi, Saftig, Paul, Blanz, Judith
Publikováno v:
Annals of Clinical & Translational Neurology; Nov2015, Vol. 2 Issue 11, p987-1001, 15p
Autor:
Dieter Hartmann, Renate Lüllmann-Rauch, R. Coenen, Volkmar Gieselmann, U. Matzner, P Dedeyn, B. Hess, Rudi D'Hooge, G. Nagels
Publikováno v:
Journal of inherited metabolic disease
Metachromatic leukodystrophy (MLD) is a lysosomal storage disorder caused by the deficiency of arylsulphatase A (ASA; EC 3.1.6.8). Deficiency of this enzyme causes intralysosomal storage of the sphingolipid cerebroside sulphate. This lipid is abundan