Enzyme replacement therapy of a novel humanized mouse model of globoid cell leukodystrophy von Matthes, Frank, Andersson, Claes, Stein, Axel, Eistrup, Carl, Fogh, Jens, Gieselmann, Volkmar, Wenger, David A., Matzner, Ulrich

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Long-term enzyme replacement therapy improves neurocognitive functioning and hippocampal synaptic plasticity in immune-tolerant alpha-mannosidosis mice von Stroobants, Stijn, Damme, Markus, Van der Jeugd, Ann, Vermaercke, Ben, Andersson, Claes, Fogh, Jens, Saftig, Paul, Blanz, Judith, D'Hooge, Rudi

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Natural history of alpha mannosidosis a longitudinal study von Beck, Michael, Olsen, Klaus J, Wraith, James E, Zeman, Jiri, Michalski, Jean-Claude, Saftig, Paul, Fogh, Jens, Malm, Dag

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Transport of Arylsulfatase A across the Blood-Brain Barrier in Vitro von Matthes, Frank, Wölte, Philipp, Böckenhoff, Annika, Hüwel, Sabine, Schulz, Mareike, Hyden, Pia, Fogh, Jens, Gieselmann, Volkmar, Galla, Hans-Joachim, Matzner, Ulrich

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Impaired Lysosomal Trimming of N-Linked Oligosaccharides Leads to Hyperglycosylation of Native Lysosomal Proteins in Mice with α-Mannosidosis von Damme, Markus, Morelle, Willy, Schmidt, Bernhard, Andersson, Claes, Fogh, Jens, Michalski, Jean-Claude, Lübke, Torben

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Enzyme replacement therapy with recombinant pro-CTSD (cathepsin D) corrects defective proteolysis and autophagy in neuronal ceroid lipofuscinosis von Marques, André R. A., Di Spiezio, Alessandro, Thießen, Niklas, Schmidt, Lina, Grötzinger, Joachim, Lüllmann-Rauch, Renate, Damme, Markus, Storck, Steffen E., Pietrzik, Claus U., Fogh, Jens, Bär, Julia, Mikhaylova, Marina, Glatzel, Markus, Bassal, Mahmoud, Bartsch, Udo, Saftig, Paul

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Analysis of cathepsin B and cathepsin L treatment to clear toxic lysosomal protein aggregates in neuronal ceroid lipofuscinosis von Di Spiezio, Alessandro, Marques, André R.A., Schmidt, Lina, Thießen, Niklas, Gallwitz, Lisa, Fogh, Jens, Bartsch, Udo, Saftig, Paul

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Induction of tolerance to human arylsulfatase A in a mouse model of metachromatic leukodystrophy von Matzner, Ulrich, Matthes, Frank, Herbst, Eva, Lüllmann-Rauch, Renate, Callaerts-Vegh, Zsuzsanna, D'Hooge, Rudi, Weigelt, Cecilia, Eistrup, Carl, Fogh, Jens, Gieselmann, Volkmar

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Recombinant pro-CTSD (cathepsin D) enhances SNCA/α-Synuclein degradation in α-Synucleinopathy models von Prieto Huarcaya, Susy, Drobny, Alice, Marques, André R. A., Di Spiezio, Alessandro, Dobert, Jan Philipp, Balta, Denise, Werner, Christian, Rizo, Tania, Gallwitz, Lisa, Bub, Simon, Stojkovska, Iva, Belur, Nandkishore R., Fogh, Jens, Mazzulli, Joseph R, Xiang, Wei, Fulzele, Amitkumar, Dejung, Mario, Sauer, Markus, Winner, Beate, Rose-John, Stefan, Arnold, Philipp, Saftig, Paul, Zunke, Friederike

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Efficacy and safety of Velmanase alfa in the treatment of patients with alpha-mannosidosis: results from the core and extension phase analysis of a phase III multicentre, double-bl... von Borgwardt, Line, Guffon, Nathalie, Amraoui, Yasmina, Dali, Christine I., De Meirleir, Linda, Gil-Campos, Mercedes, Heron, Bénédicte, Geraci, Silvia, Ardigò, Diego, Cattaneo, Federica, Fogh, Jens, Van den Hout, J. M. Hannerieke, Beck, Michael, Jones, Simon A., Tylki-Szymanska, Anna, Haugsted, Ulla, Lund, Allan M.

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Intracerebroventricular enzyme infusion corrects central nervous system pathology and dysfunction in a mouse model of metachromatic leukodystrophy von Stroobants, Stijn, Gerlach, Debora, Matthes, Frank, Hartmann, Dieter, Fogh, Jens, Gieselmann, Volkmar, D'Hooge, Rudi, Matzner, Ulrich

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Efficacy of enzyme replacement therapy in an aggravated mouse model of metachromatic leukodystrophy declines with age von MATTHES, Frank, STROOBANTS, Stijn, GERLACH, Debora, WOHLENBERG, Claudia, WESSIG, Carsten, FOGH, Jens, GIESELMANN, Volkmar, ECKHARDT, Matthias, D'HOOGE, Rudi, MATZNER, Ulrich

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Comprehensive long-term efficacy and safety of recombinant human alpha-mannosidase (velmanase alfa) treatment in patients with alpha-mannosidosis von Lund, Allan M., Borgwardt, Line, Cattaneo, Federica, Ardigò, Diego, Geraci, Silvia, Gil-Campos, Mercedes, De Meirleir, Linda, Laroche, Cécile, Dolhem, Philippe, Cole, Duncan, Tylki-Szymanska, Anna, Lopez-Rodriguez, Monica, Guillén-Navarro, Encarna, Dali, Christine I., Héron, Bénédicte, Fogh, Jens, Muschol, Nicole, Phillips, Dawn, Van den Hout, J. M. Hannerieke, Jones, Simon A., Amraoui, Yasmina, Harmatz, Paul, Guffon, Nathalie

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Investigating surrogate cerebrospinal fluid matrix compositions for use in quantitative LC-MS analysis of therapeutic antibodies in the cerebrospinal fluid von Fogh, Jens Rose, Jacobsen, Anne-Marie, Nguyen, Tam T. T. N., Rand, Kasper D., Olsen, Line Rørbæk

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Alpha-mannosidosis: Correlation between phenotype, genotype and mutant MAN2B1 subcellular localisation Inherited metabolic diseases von Borgwardt, Line, Stensland, Hilde Monica Frostad Riise, Olsen, Klaus Juul, Wibrand, Flemming, Klenow, Helle, Beck, Michael, Amraoui, Yasmina, Arash, Laila, Fogh, Jens, Nilssen, Øivind, Lund, Allan Meldgaard

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Alpha-mannosidosis: correlation between phenotype, genotype and mutant MAN2B1 subcellular localisation von Borgwardt, Line, Stensland, Hilde Monica Frostad Riise, Olsen, Klaus Juul, Wibrand, Flemming, Klenow, Helle Bagterp, Beck, Michael, Amraoui, Yasmina, Arash, Laila, Fogh, Jens, Nilssen, Øivind, Dali, Christine I, Lund, Allan Meldgaard

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Enzyme Replacement Improves Ataxic Gait and Central Nervous System Histopathology in a Mouse Model of Metachromatic Leukodystrophy von Matzner, Ulrich, Lüllmann-Rauch, Renate, Stroobants, Stijn, Andersson, Claes, Weigelt, Cecilia, Eistrup, Carl, Fogh, Jens, D'Hooge, Rudi, Gieselmann, Volkmar

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Enzyme replacement improves nervous system pathology and function in a mouse model for metachromatic leukodystrophy von Matzner, Ulrich, Herbst, Eva, Hedayati, Kerstin Khalaj, Lüllmann-Rauch, Renate, Wessig, Carsten, Schröder, Stephan, Eistrup, Carl, Möller, Christer, Fogh, Jens, Gieselmann, Volkmar

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Reversal of peripheral and central neural storage and ataxia after recombinant enzyme replacement therapy in α-mannosidosis mice von Blanz, Judith, Stroobants, Stijn, Lüllmann-Rauch, Renate, Morelle, Willy, Lüdemann, Meike, D'Hooge, Rudi, Reuterwall, Helena, Michalski, Jean Claude, Fogh, Jens, Andersson, Claes, Saftig, Paul

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Non-inhibitory antibodies impede lysosomal storage reduction during enzyme replacement therapy of a lysosomal storage disease von Matzner, Ulrich, Matthes, Frank, Weigelt, Cecilia, Andersson, Claes, Eistrup, Carl, Fogh, Jens, Gieselmann, Volkmar

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