Few, if any people, want to live to 120 years old if they have to spend their last 20 years in a nursing home, bedbound, not recognizing their family or caretakers. If that’s what anti-aging strategies bring, most of us would pass. That’s not what anti-aging research is about fortunately. We aim to provide a productive longer life where you die at an advanced age with “your boots on.”
Just like we can learn about memory and cognition from studying Alzheimer’s and Parkinson’s disease, we can look at connective tissue diseases like Progeria, also known as Hutchinson-Gilford Progeria Syndrome (HGPS) and Werner’s Syndrome (adult Progeria) to understand how connective tissue breakdown dramatically shortens life.
On September 10, 2010, ABC’s 20/20 aired a 1-hour program on Progeria entitled, When Seven Looks Like 70…A Race Against Time for Three Young Girls.
The data refers to three clinical studies demonstrating the relationship. One example is the disease known as Progeria syndrome, which causes those children affected to age at an extremely rapid rate. Patients with Hutchinson-Gilford progeria syndrome (HGPS) develop accelerated atherosclerosis of the cerebral and coronary arteries. Unlike arteriosclerosis in the general population, however, in progeria, the only lipid abnormality is decreased high-density lipoprotein cholesterol levels.
Patients with HGPS also develop other clinical signs of accelerated aging, including loss of subcutaneous fat and muscle, skin atrophy, osteoporosis, arthritis, poor growth, and alopecia (hair loss). Interestingly, patients with HGPS do not develop other disease processes associated with aging, such as increased tumor formation, cataract development, or senility. In this sense, HGPS is considered a segmental progeroid syndrome in that it does not recapitulate all of the characteristic phenomena of aging.Extensive lipofuscin (age spot) deposition, a marker for aging, is extensively distributed in patients with HGPS. Affected organs include the kidneys, brain, adrenal glands, liver, testes, and heart.
A characteristic finding in persons with progeria is an increase in hyaluronic acid excretion. In addition to persons with progeria, it is only detected in those with Werner Syndrome, a disease characterized by a later onset of premature aging that occurs during the second decade of life.
Usually, hyaluronic acid and other glycosaminoglycan production increases during the fifth to seventh decades of life. Possibly, the increase in hyaluronic acid is a normal feature of advancing age. Fibroblasts from patients with progeria show a 3-fold increase in total glycosaminoglycan production and, in particular, hyaluronic acid production, compared with age-matched control groups. This increase results from an abnormality in degradation and is not caused by increased synthesis. Data from embryonic development suggest that changes in the level of hyaluronic acid are extremely important for morphological development. Experiments performed in chick embryos have demonstrated a correlation between cell differentiation and hyaluronic acid degradation. Hyaluronic acid is also necessary for the morphologic development of blood vessels in chick embryos. A reduction or absence of blood vessels is noted in regions of high hyaluronic acid levels. The decreased density of vasculature, sclerodermatous changes in the skin, and the high prevalence of cardiovascular disease present in persons with progeria may be induced by increased hyaluronic acid levels. Increased hyaluronic acid levels may also promote calcification of blood vessels, thus contributing to arteriosclerosis.