CHRU Nancy - Centre Hospitalier Régional Universitaire de Nancy (Siège social : 29 avenue du Maréchal de Lattre de Tassigny, CO 60034, 54035 NANCY CEDEX
Hôpital Central : 29 Avenue du Maréchal de Lattre de Tassigny, 54035 Nancy
Hôpitaux de Brabois / Enfants : Rue du Morvan, 54500 Vandœuvre-lès-Nancy
Maternité régionale :10 Avenue Dr Heydenreich, 54000 Nancy - France)
CHRU Nancy - Centre Hospitalier Régional Universitaire de Nancy (Siège social : 29 avenue du Maréchal de Lattre de Tassigny, CO 60034, 54035 NANCY CEDEX
Hôpital Central : 29 Avenue du Maréchal de Lattre de Tassigny, 54035 Nancy
Hôpitaux de Brabois / Enfants : Rue du Morvan, 54500 Vandœuvre-lès-Nancy
Maternité régionale :10 Avenue Dr Heydenreich, 54000 Nancy - France)
Abstract : Pseudoxanthoma elasticum is an inherited metabolic disorder resulting from ABCC6 gene mutations. It is characterized by progressive calcification and fragmentation of elastic fibers in the skin, retina, and the arterial wall. Despite calcium accumulation in the arteries of patients with pseudoxanthoma elasticum, functional consequences remain unknown. In the present study, we investigated arterial structure and function in Abcc6(-/-) mice, a model of the human disease.
https://hal.univ-lorraine.fr/hal-01738627
Contributor : Dcac Ul <>
Submitted on : Tuesday, March 20, 2018 - 4:23:05 PM Last modification on : Tuesday, October 13, 2020 - 10:47:44 AM
G. Kauffenstein, A. Pizard, Y. Le Corre, E. Vessieres, L Grimaud, et al.. Disseminated Arterial Calcification and Enhanced Myogenic Response Are Associated With Abcc6 Deficiency in a Mouse Model of Pseudoxanthoma Elasticum. Arteriosclerosis, Thrombosis, and Vascular Biology, American Heart Association, 2014, 34 (5), pp.1045 - 1056. ⟨10.1161/ATVBAHA.113.302943⟩. ⟨hal-01738627⟩