Lentiviral gene rescue of a Bernard-Soulier mouse model to study platelet glycoprotein Ibβ function.
Fiche publication
Date publication
juillet 2016
Journal
Journal of thrombosis and haemostasis : JTH
Auteurs
Membres identifiés du Cancéropôle Est :
Dr GACHET Christian, Dr MANGIN Pierre
Tous les auteurs :
Strassel C, Bull A, Moog S, Receveur N, Mallo L, Mangin P, Eckly A, Freund M, Dubart-Kupperschmitt A, Gachet C, Lanza F
Lien Pubmed
Résumé
Essentials A signaling role of glycoprotein (GP)Ibβ is postulated but not formally demonstrated in platelets. Lentiviral-mediated rescue in knock-out mice can be used to evaluate GPIbβ function in vivo. Transduction of the native subunit corrected the main defects associated with GPIb-IX deficiency Deletion of intracellular 159-170 segment increased thrombosis, 150-160 removal increased bleeding.
Mots clés
Animals, Aorta, metabolism, Bernard-Soulier Syndrome, genetics, Bleeding Time, Blood Platelets, metabolism, Bone Marrow Cells, cytology, Female, Gene Deletion, Gene Expression Regulation, Gene Transfer Techniques, Genetic Vectors, HEK293 Cells, Hemorrhage, Hemostasis, Humans, Lentivirus, Male, Megakaryocytes, cytology, Mice, Mice, Knockout, Microscopy, Electron, Scanning, Platelet Glycoprotein GPIb-IX Complex, metabolism, Protein Domains, Thrombosis, metabolism, Transgenes, von Willebrand Factor, metabolism
Référence
J. Thromb. Haemost.. 2016 07;14(7):1470-9