TY - JOUR AU - Villa-Bellosta, Ricardo AU - Rivera-Torres, Jose AU - Osorio, Fernando G AU - Acin-Perez, Rebeca AU - Enriquez, Jose Antonio AU - López-Otín, Carlos AU - Andres, Vicente PY - 2013 DO - 10.1161/CIRCULATIONAHA.112.000571 SN - 0009-7322 UR - http://hdl.handle.net/20.500.12105/7754 AB - BACKGROUND: Progerin is a mutant form of lamin A responsible for Hutchinson-Gilford progeria syndrome (HGPS), a premature aging disorder characterized by excessive atherosclerosis and vascular calcification that leads to premature death, predominantly... LA - eng KW - ATP KW - Hutchinson-Gilford progeria syndrome KW - muscle KW - Progerin KW - Pyrophosphate KW - Tissue-non specific alkaline phosphatase KW - Vascular calcification KW - Smooth KW - Adenosine Triphosphate KW - Alkaline Phosphatase KW - Animals KW - Aorta KW - Cells, Cultured KW - Diphosphates KW - Disease Models, Animal KW - Lamin Type A KW - Male KW - Mice KW - Mice, Inbred C57BL KW - Mice, Mutant Strains KW - Mitochondria, Muscle KW - Muscle, Smooth, Vascular KW - Progeria KW - Treatment Outcome KW - Vascular Calcification TI - Defective extracellular pyrophosphate metabolism promotes vascular calcification in a mouse model of Hutchinson-Gilford progeria syndrome that is ameliorated on pyrophosphate treatment TY - journal article ER -