Problem

Premature aging

Other Names:
Premature ageing
Nature:

Progeroid syndromes (PS) are a group of rare genetic disorders which mimic physiological aging, making affected individuals appear to be older than they are. The term progeroid syndrome does not necessarily imply progeria (Hutchinson–Gilford progeria syndrome), which is a specific type of progeroid syndrome.

Progeroid means "resembling premature aging", a definition that can apply to a broad range of diseases. Familial Alzheimer's disease and familial Parkinson's disease are two well-known accelerated-aging diseases that are more frequent in older individuals. They affect only one tissue and can be classified as unimodal progeroid syndromes. Segmental progeria, which is more frequently associated with the term progeroid syndrome, tends to affect multiple or all tissues while causing affected individuals to exhibit only some of the features associated with aging.

All disorders within this group are thought to be monogenic, meaning they arise from mutations of a single gene. Most known PS are due to genetic mutations that lead to either defects in the DNA repair mechanism or defects in lamin A/C.

Examples of PS include Werner syndrome (WS), Bloom syndrome (BS), Rothmund–Thomson syndrome (RTS), Cockayne syndrome (CS), xeroderma pigmentosum (XP), trichothiodystrophy (TTD), combined xeroderma pigmentosum-Cockayne syndrome (XP-CS), restrictive dermopathy (RD), and Hutchinson–Gilford progeria syndrome (HGPS). Individuals with these disorders tend to have a reduced lifespan. Progeroid syndromes have been widely studied in the fields of aging, regeneration, stem cells, and cancer. The most widely studied of the progeroid syndromes are Werner syndrome and Hutchinson–Gilford progeria, as they are seen to most resemble natural aging.

Incidence:
It has been proven that there is one gene that causes a disease called Hutchinson-Gilford progeria. It makes children age so fast that by 5 they lose their hair, become wrinkled and develop cardiovascular disease. They look like little old men and women. And they die at about 12. A different gene causes Huntington's disease. Symptoms begin when people are in their 30s or 40s, after they have finished reproducing. There are only a few dozen children in the world with progeria. There are tens of thousands of people with Huntington's disease.
Broader Problems:
Human ageing
Reduced By:
Excessive longevity
Values:
Prematurity
Problem Type:
G: Very specific problems
Subject(s):
Society Elderly
Related UN Sustainable Development Goals:
GOAL 3: Good Health and Well-being
Date of last update
09.03.2000 – 00:00 CET