Progeria and accelerated cardiovascular aging

Abstract

Hutchinson–Gilford Progeria syndrome (HGPS) is characterized by accelerated aging leading to death in the teen years, usually due to severe coronary and/or carotid disease. The clinical presentation includes stunted growth, alopecia, loss of subcutaneous fat, osteoporosis, and cardiovascular disorders occurring in late childhood and the teen years including hypertension and accelerated vascular aging that precipitates myocardial infarction and cerebrovascular attacks. The disease is a nuclear laminopathy, due to a Lmna gene mutation. The aberrant protein (Progerin) accumulates in and distorts the nuclear envelope. We review the genetic and biochemical mechanism of HGPS; the clinical presentation with special attention to the cardiovascular pathology and complications; current therapeutic developments to address the disease; and the results of the clinical trials attempting to translate basic research insights into therapeutic benefit. An understanding of HGPS may lead to better treatment of other age-related disorders, particularly cardiovascular diseases.

Keywords

• Atherosclerosis
• cardiovascular aging
• cardiovascular disease
• nuclear lamina
• progeria
• progerin
• telomere