Progeria: An Introduction
Progeria is a very rare genetic condition which occurs one in several million people. The children affected by it, seem to be look much aged, than they really are. It was first described by Johnathan Hutchinson and Hastings Gilford, the former in 1886 and latter, a bit later in 1897.
The disorder is hence also called as Hutchinson-Gilford Progeria Syndrome (HGPS). Progeria comes from the greek word "geras".
People affected by the disorder tend to live till the age of thirteen, but some do live into their late teens and early twenties and some have been reported to have even lived up till their forties. The disorder is generally not hereditary, though there is a unique heritable form.
It is a genetic condition, that occurs as a new mutation. It has garnered a lot of attention in the scientific community, because of the opportunity for scientists to study the process of aging.
Any disease with symptoms resembling accelerated aging process is referred to as Progeria. It is caused by a mutation in a protein called Lamin A, which results in a misshapen cell nucleus. It is now believed to result from a gene mutation, arising around the time of conception or shortly thereafter.
The Progeria Research Foundation is a non-profit organization in the United States devoted to discovering a cure for progeria. Since heart disease is a leading cause of death worldwide, developing a cure for progeria may have applications beyond ending the suffering and increasing the lifespan of affected children. In addition, defects in Lamin A may be responsible for normal aging as well as for the accelerated aging symptoms of progeria.
Hutchinson-Gilford progeria syndrome is considered an autosomal dominant condition, which means one copy of the altered gene in each cell is sufficient to cause the disorder. The condition results from new mutations in the LMNA gene, and almost always occurs in people with no history of the disorder in their family