What is the origin and effects of Progeria?

Progeria is a very rare disorder, occuring in about 1 in 8
million births. It is a disease which causes extremely rapid aging of the body,
particularly of the heart. It is usually diagnosed in early childood, and the victims
rarely survive into their teens.

While progeria is a
genetic disease, it is not passed down in families. It is caused by a spontaneous
mutation of the LMNA gene, which codes for the protein Lamin-A. Lamin-A creates the
structure of the cell nucleus. Children born with this mutation are unable to make
Lamin-A correctly, so their cell nuclei become unstable, and do not maintain or repair
themselves. As a result, the normal wear and tear in their bodies does not heal, and so
accumulates at a very rapid rate. Every body system is affected by this; some of the
more obvious symptoms are skeletal abnormalities, hair loss, thin, aged-looking skin,
and very little body fat. Most progeria sufferers die of heart
failure.

At this time there is no treatment that is
effective for progeria. The main interventions involved psychological support for the
victim and their family.