Progeria (Hutchinson-Gilford Progeria Syndrome ("Progeria", or "HGPS") is a rare, fatal genetic condition characterized by an appearance of accelerated aging in children. Its name is derived from the Greek and means "prematurely old." While there are different forms of Progeria*, the classic type is Hutchinson-Gilford Progeria Syndrome, which was named after the doctors who first described it in England; in 1886 by Dr. Jonathan Hutchinson and in 1897 by Dr. Hastings Gilford.
HGPS is caused by a mutation in the gene
called LMNA (pronounced, lamin - a). The LMNA gene produces the Lamin A
protein, which is the structural scaffolding that holds the nucleus of a cell
together. Researchers now believe that the defective Lamin A protein makes the
nucleus unstable. That cellular instability appears to lead to the process of
premature aging in Progeria.
Although they are born looking healthy,
children with Progeria begin to display many characteristics of accelerated
aging at around 18-24 months of age. Progeria signs include growth failure,
loss of body fat and hair, aged-looking skin, stiffness of joints, hip
dislocation, generalized atherosclerosis, cardiovascular (heart) disease and
stroke. The children have a remarkably similar appearance, despite differing
ethnic backgrounds. Children with Progeria die of atherosclerosis (heart disease) at an average age of thirteen years.
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