Search Results - "Matzner, U"

Bone marrow stem cell-based gene transfer in a mouse model for metachromatic leukodystrophy: effects on visceral and nervous system disease manifestations by MATZNER, U, HARTMANN, D, LÜLLMANN-RAUCH, R, COENEN, R, ROTHERT, F, MANSSON, J.-E, FREDMAN, P, D'HOOGE, R, DE DEYN, P. P, GIESELMANN, V

“…Arylsulfatase A (ASA) knockout mice represent an animal model for the lysosomal storage disease metachromatic leukodystrophy (MLD). Stem cell gene therapy with…”
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Bone marrow stem cell-based gene transfer in a mouse model for metachromatic leukodystrophy: effects on visceral and nervous system disease manifestations by Matzner, U, Hartmann, D, Luellmann-Rauch, R, Coenen, R, Rothert, F, Maensson, J-E, Fredman, P, D'Hooge, R, De Deyn, PP, Gieselmann, V

“…Arylsulfatase A (ASA) knockout mice represent an animal model for the lysosomal storage disease metachromatic leukodystrophy (MLD). Stem cell gene therapy with…”
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Retrovirally expressed human arylsulfatase A corrects the metabolic defect of arylsulfatase A-deficient mouse cells by MATZNER, U, HABETHA, M, GIESELMANN, V

“…A deficiency of arylsulfatase A (ASA) causes the lysosomal storage disease metachromatic leukodystrophy (MLD) which is characterized primarily by demyelination…”
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Long-term expression and transfer of arylsulfatase A into brain of arylsulfatase A-deficient mice transplanted with bone marrow expressing the arylsulfatase A cDNA from a retroviral vector by MATZNER, U, HARZER, K, LEARISH, R. D, BARRANGER, J. A, GIESELMANN, V

“…A deficiency of arylsulfatase A (ASA) results in the lysosomal lipid storage disease metachromatic leukodystrophy. The disease mainly affects the central…”
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Lysosomal sulfoglycolipid storage in the kidneys of mice deficient for arylsulfatase A (ASA) and of double-knockout mice deficient for ASA and galactosylceramide synthase by Lüllmann-Rauch, R, Matzner, U, Franken, S, Hartmann, D, Gieselmann, V

“…The inherited deficiency of arylsulfatase A (ASA) causes lysosomal accumulation of sulfoglycolipids (mainly sulfo-galactosylceramide, S-GalCer ) and leads to…”
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Metachromatic leukodystrophy: Molecular genetics and an animal model by Gieselmann, V., Matzner, U., Hess, B., Lüllmann‐Rauch, R., Coenen, R., Hartmann, D., D'Hooge, R., Dedeyn, P., Nagels, G.

“…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…”
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Modest phenotypic improvements in ASA-deficient mice with only one UDP-galactose:ceramide-galactosyltransferase gene by Franken, S, Wittke, D, Mansson, J E, D'Hooge, R, De Deyn, P P, Lüllmann-Rauch, R, Matzner, U, Gieselmann, V

“…Arylsulfatase A (ASA)-deficient mice are a model for the lysosomal storage disorder metachromatic leukodystrophy. This lipidosis is characterised by the…”
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Bone marrow stem cell gene therapy of arylsulfatase A-deficient mice, using an arylsulfatase A mutant that is hypersecreted from retrovirally transduced donor-type cells by Matzner, U, Schestag, F, Hartmann, D, Lüllmann-Rauch, R, D'Hooge, R, De Deyn, P P, Gieselmann, V

“…Arylsulfatase A (ASA)-deficient mice represent an animal model for the fatal lysosomal storage disease metachromatic leukodystrophy, which is characterized by…”
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Expression of the two mannose 6-phosphate receptors is spatially and temporally different during mouse embryogenesis by MATZNER, U, VON FIGURA, K, POHLMANN, R

“…Mammalian cells express two mannose 6-phosphate receptors, MPR46 and MPR300, both of which mediate the targeting of lysosomal enzymes to lysosomes…”
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Targeted disruption of the M(r) 46,000 mannose 6‐phosphate receptor gene in mice results in misrouting of lysosomal proteins by Köster, A., Saftig, P., Matzner, U., Figura, K., Peters, C., Pohlmann, R.

“…Lysosomal enzymes containing mannose 6‐phosphate recognition markers are sorted to lysosomes by mannose 6‐phosphate receptors (MPRs). The physiological…”
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Intracerebroventricular enzyme infusion corrects central nervous system pathology and dysfunction in a mouse model of metachromatic leukodystrophy by Stroobants, Stijn, Gerlach, Debora, Matthes, Frank, Hartmann, Dieter, Fogh, Jens, Gieselmann, Volkmar, D'Hooge, Rudi, Matzner, Ulrich

“…Arylsulfatase A (ASA) catalyzes the desulfation of sulfatide, a major lipid component of myelin. Inherited functional deficiencies of ASA cause the lysosomal…”
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Efficacy of enzyme replacement therapy in an aggravated mouse model of metachromatic leukodystrophy declines with age by MATTHES, Frank, STROOBANTS, Stijn, GERLACH, Debora, WOHLENBERG, Claudia, WESSIG, Carsten, FOGH, Jens, GIESELMANN, Volkmar, ECKHARDT, Matthias, D'HOOGE, Rudi, MATZNER, Ulrich

“…Metachromatic leukodystrophy (MLD) is a lysosomal storage disease caused by a functional deficiency of arylsulfatase A (ASA). Previous studies in ASA-knockout…”
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Translational control of arylsulfatase A expression in mouse testis by Kreysing, J, Polten, A, Lukatela, G, Matzner, U, von Figura, K, Gieselmann, V

“…Arylsulfatase A is a lysosomal enzyme that is involved in the degradation of sulfated glycolipids. High levels of arylsulfatase A mRNA are found in germ cells…”
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Enzyme replacement improves nervous system pathology and function in a mouse model for metachromatic leukodystrophy by Matzner, Ulrich, Herbst, Eva, Hedayati, Kerstin Khalaj, Lüllmann-Rauch, Renate, Wessig, Carsten, Schröder, Stephan, Eistrup, Carl, Möller, Christer, Fogh, Jens, Gieselmann, Volkmar

“…A deficiency of arylsulfatase A (ASA) causes the lysosomal storage disease metachromatic leukodystrophy, which is characterized by accumulation of the…”
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Gene therapy of metachromatic leukodystrophy by Matzner, Ulrich, Gieselmann, Volkmar

“…Metachromatic leukodystrophy (MLD) is a lysosomal storage disease that is caused by a deficiency of arylsulfatase A (ASA). The deficiency results in the…”
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Secretion of phosphomannosyl-deficient arylsulphatase A and cathepsin D from isolated human macrophages by Muschol, Nicole, Matzner, Ulrich, Tiede, Stephan, Gieselmann, Volkmar, Ullrich, Kurt, Braulke, Thomas

“…The transfer of macrophage-secreted arylsulphatase A (ASA) to enzyme-deficient brain cells is part of the therapeutic concept of bone marrow transplantation in…”
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Gene therapy: prospects for glycolipid storage diseases by Gieselmann, Volkmar, Matzner, Ulrich, Klein, Diana, Eric Mansson, Jan, D'Hooge, Rudi, DeDeyn, Peter D., Lüllmann Rauch, Renate, Hartmann, Dieter, Harzer, Klaus

“…gene therapy. We review the rationale for gene therapy in lysosomal disorders and present data, in particular, about trials in an animal model of metachromatic…”
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Quantitative genotyping of single-nucleotide polymorphisms by allele-specific oligonucleotide hybridization on DNA microarrays by Rickert, Andreas M., Ballvora, Agim, Matzner, Ulrich, Klemm, Manfred, Gebhardt, Christiane

“…Genotyping of SNPs (single‐nucleotide polymorphisms) has challenged the development of several novel techniques [Twyman and Primrose (2003) Pharmacogenomics 4,…”
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Targeted disruption of the Mr 46 000 mannose 6-phosphate receptor gene in mice results in misrouting of lysosomal proteins by KÖSTER, A, SAFTIG, P, MATZNER, U, VON FIGURA, K, PETERS, C, POHLMANN, R

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Expression of mannose 6‐phosphate receptors in chicken by Matzner, Ulrich, Hille‐Rehfeld, Annette, Figura, Kurt Von, Pohlmann, Regina

“…In mammals, the sorting of newly synthesized lysosomal enzymes is accomplished by two mannose 6‐phosphate receptors (MPR) designated MPR46 and MPR300. MPR300…”
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