Premature Aging in Children: The Curse of Progeria

Progeria or Hutchinson-Gilford (Progeria) syndrome (HGPS) is a genetic "disease" characterized by the following signs:

• Accelerated aging of the affected individual starting from around 1-4 years of age

• Growth failure resulting in a short stature

• Gradual loss of hair (balding) and body fat

• Aging skin

• Stiffness of joints

• Hip dislocation

• Atherosclerosis

• Cardiovascular problems and strokes

• Macrocephaly

• Small jaw

• Dental problems

Progeria is an extremely rare condition. There are only an estimated 40 cases worldwide. The word Progeria comes from the Greek word "progeros" meaning "prematurely old".

The cause for this condition has been pinpointed to the mutation of a gene called LMNA (lamin A protein) on chromosome 1. the mutated form of lamin A is commonly known as progerin.

This gene produces a protein by the name of Lamin A which acts as the structural scaffolding that holds the nucleus of a cell together. The mutant form of Lamin A builds up in the nucleus of cells. The nucleus becomes deformed and growth slows and stops. It particularly affects cells in blood vessels.

Strangely enough, Progeria does in some way teach us about the normal processes of aging.

The most common cause of death in children with Progeria is atherosclerosis. This can be defined as a disease where plaque builds up and causes hardening and narrowing in arteries.

The restricted blood flow may lead to death through heart attacks or strokes. Most children with Progeria do not live past their early teens.

Heart attacks and atherosclerosis are also leading causes of death in the elderly and researchers believe that finding a cure for Progeria may benefit millions of other people.

Dr Leslie Gordon, The Progeria Research Foundation's Medical Director was senior author of the study, Arteriosclerosis, Thrombosis, and Vascular Biologym.

In August 2010, he made this comment: "By examining one of the rarest diseases in the world, we are gaining crucial insight into a disease that affects millions of people worldwide. Ongoing research has the potential to have a significant impact on our understanding of heart disease and aging."