Defective Extracellular Pyrophosphate Metabolism Promotes Vascular Calcification in a Mouse Model of Hutchinson-Gilford Progeria Syndrome that is Ameliorated upon Pyrophosphate Treatment

نویسندگان

  • José Rivera
  • Fernando G. Osorio
  • Rebeca Acín-Pérez
  • José A. Enriquez
  • Carlos López-Otín
چکیده

Background—Progerin is a mutant form of lamin A responsible for Hutchinson-Gilford progeria

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منابع مشابه

Defective extracellular pyrophosphate metabolism promotes vascular calcification in a mouse model of Hutchinson-Gilford progeria syndrome that is ameliorated on pyrophosphate treatment.

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 of myocardial infarction or stroke. The goal of this study was to investigate mechanisms that cause excessive vascular calcification in HGPS. ME...

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A Case Report of Hutchinson-Gilford Progeria Syndrome

Hutchinson-Gilford Progeria Syndrome (HGPS), a rare genetic condition occurs one in every 8 million live births. HGPS is characterized by premature aging in various organs. The average survival rate of the affected patients is 13 years, and their most common causes of death are myocardial infarction and stroke. This disease occurs due to a gene mutation in the chromosomes of the patient. There ...

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تاریخ انتشار 2013