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Cardiovascular pathology in Hutchinson-Gilford progeria: correlation with the vascular pathology of aging

Michelle Olive1, Ingrid Harten, Richard Mitchell

  • 1National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD, USA.

Arteriosclerosis, Thrombosis, and Vascular Biology
|August 28, 2010
PubMed

Insights

Hutchinson-Gilford progeria syndrome (HGPS) accelerates cardiovascular disease (CVD) and shares pathologies with aging. Progerin accumulation in blood vessels increases with age, suggesting its role in common cardiovascular aging.

Area of Science:

  • Cardiovascular Pathology
  • Gerontology
  • Rare Genetic Disorders

Background:

  • Hutchinson-Gilford progeria syndrome (HGPS) causes rapid cardiovascular disease (CVD) in children.
  • HGPS patients often die from myocardial infarction or stroke at a young age.

Purpose of the Study:

  • To conduct the first histological comparison of cardiovascular tissues between HGPS and aging-related CVD.
  • To investigate the role of progerin in HGPS and general cardiovascular aging.

Main Methods:

  • Structural and immunohistological analysis of cardiovascular tissues from two HGPS patients.
  • Comparative analysis with tissues from non-HGPS individuals across a wide age range.

Main Results:

  • HGPS cardiovascular tissues showed atherosclerosis, arteriolosclerosis, and prominent adventitial fibrosis.
  • Progerin was detected in HGPS coronary arteries and also present in non-HGPS individuals.
  • Progerin staining increased significantly with age in coronary arteries (3.34% per year).

Conclusions:

  • Cardiovascular pathology in HGPS shares similarities with aging-related CVD.
  • HGPS exhibits more pronounced adventitial fibrosis than typical CVD.
  • Increasing vascular progerin with age suggests a role in general population cardiovascular aging.
Abstract

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