20110801

Progeria

Progeria is an extremely trouble genetic condition that is remarkable because its symptoms strongly resemble normal human aging, but occur in young children or symptoms resembling aspects of aging are manifested at an early age.Ninety percent of children with progeria have a mutation on the gene that encodes the protein lamin A. Progeria usually occurs without cause. It is only very rarely seen in more than one child in a family.The word Progeria comes from the Greek words "pro" means "before" and "géras" means "old age". The disorder has very low incidences and occurs in an estimated 1 per 8 million live births.Those born with progeria typically live to their mid teens and early twenties.

It is a genetic condition that occurs as a new mutation (de novo), and is not inherited. Although the term progeria applies strictly speaking to all diseases characterized by premature aging symptoms, and is often used as such, it is often applied specifically in reference to Hutchinson-Gilford Progeria Syndrome.

Scientists are particularly interested in progeria because it might reveal clues about the normal process of aging.Progeria was first described in 1886 by Jonathan Hutchinson.It was also described independently in 1897 by Hastings Gilford.The condition was later named Hutchinson-Gilford Progeria Syndrome (HGPS).