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Structure of NPP1, an Ectonucleotide Pyrophosphatase/Phosphodiesterase Involved in Tissue Calcification
Authors:Silvia Jansen  Anastassis Perrakis  Chris Ulens  Claudia Winkler  Maria Andries  Robbie P Joosten  Maarten Van Acker  Frank P Luyten  Wouter H Moolenaar  Mathieu Bollen
Institution:1. Laboratory of Biosignaling and Therapeutics, Department of Cellular and Molecular Medicine, University of Leuven, 3000 Leuven, Belgium;2. Laboratory of Structural Neurobiology, Department of Cellular and Molecular Medicine, University of Leuven, 3000 Leuven, Belgium;3. Laboratory for Skeletal Development and Joint Disorders, University of Leuven, 3000 Leuven, Belgium;4. Division of Biochemistry, The Netherlands Cancer Institute, 1066CX Amsterdam, The Netherlands;5. Division of Cell Biology, The Netherlands Cancer Institute, 1066CX Amsterdam, The Netherlands
Abstract:
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  • Highlights? We determine the crystal structure of mouse NPP1 ? We elucidate the mechanism of NPP1 dimer formation and secretion ? The structure explains the dramatic functional differences between NPP1 and NPP2 ? The structure allows defining the impact of pathogenic mutations in human NPP1
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