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Ursula Lüthi
Researcher at University of Zurich
Publications - 8
Citations - 1557
Ursula Lüthi is an academic researcher from University of Zurich. The author has contributed to research in topics: Anchoring fibrils & Endosome. The author has an hindex of 8, co-authored 8 publications receiving 1506 citations.
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Journal ArticleDOI
Correction of X-linked chronic granulomatous disease by gene therapy, augmented by insertional activation of MDS1-EVI1, PRDM16 or SETBP1.
Marion Ott,Manfred Schmidt,Kerstin Schwarzwaelder,Stefan Stein,Ulrich Siler,Ulrike Koehl,Hanno Glimm,K. Kühlcke,Andrea Schilz,Hana Kunkel,Sonja Naundorf,Andrea Brinkmann,Annette Deichmann,Marlene Fischer,Claudia R. Ball,Ingo H. Pilz,Cynthia E. Dunbar,Yang Du,Nancy A. Jenkins,Neal G. Copeland,Ursula Lüthi,Moustapha Hassan,Adrian J. Thrasher,Dieter Hoelzer,Christof von Kalle,Reinhard Seger,Manuel Grez +26 more
TL;DR: The results suggest that gene therapy in combination with bone marrow conditioning can be successfully used to treat inherited diseases affecting the myeloid compartment such as CGD.
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Cross-linking of the dermo-epidermal junction of skin regenerating from keratinocyte autografts. Anchoring fibrils are a target for tissue transglutaminase.
Michael Raghunath,Bianca Höpfner,Daniel Aeschlimann,Ursula Lüthi,Martin Meuli,Stefan Altermatt,Rita Gobet,Leena Bruckner-Tuderman,Beat Steinmann +8 more
TL;DR: Tissue transglutaminase appears to play an important role not only in cross-linking of the papillary dermis but also of the dermo-epidermal junction in particular in regenerating skin.
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Three Novel Homozygous Point Mutations and a New Polymorphism in the COL17A1 Gene: Relation to Biological and Clinical Phenotypes of Junctional Epidermolysis Bullosa
Hauke Schumann,Nadja Hammami-Hauasli,Leena Pulkkinen,Alain Mauviel,Wolfgang Küster,Ursula Lüthi,Katsushi Owaribe,Jouni Uitto,Leena Bruckner-Tuderman +8 more
TL;DR: The homozygous nonsense mutations in the COL17A1 gene were consistent with the absence of the collagen from the skin and with the GABEB phenotype, whereas homozygosity for the missense mutation resulted in expression of aberrant collagen XVII and, clinically, in localisata JEB.
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Some, but not all, glycine substitution mutations in col7a1 result in intracellular accumulation of collagen vii, loss of anchoring fibrils, and skin blistering
Nadja Hammami-Hauasli,Hauke Schumann,Michael Raghunath,Oliver Kilgus,Ursula Lüthi,Thomas A. Luger,Leena Bruckner-Tuderman +6 more
TL;DR: It is demonstrated that collagen VII presents a remarkable exception among collagens in that not all glycine substitutions within the triple helix exert dominant-negative interference and that the biological consequences of the substitutions probably depend on their position within the double helix.
Journal ArticleDOI
Low-temperature fluorography induced by tritium-labelled compounds on thin- layer chromatograms
Ursula Lüthi,P. G. Waser +1 more
TL;DR: A specific contribution of the cell genome to one of the early steps during the multiplication of influenza viruses is unlikely and there is no need for such an assumption, since the inhibitory effect of agents reacting with thecell genome might be explained by a destruction of the influenza genome in the cell nucleus without influencing the NDV multiplication in the cytoplasm.