Monday, May 21, 2012

comparison of the progerin infeected cell and a normal cell

Fig. 2. Western blots showed differentially expressed proteins in HGPS cell lines. P1: progeria cell line HGADFN127, P2: progeria cell line AG11498, P3: progeria cell line AG01972; N1: normal control cell line C10, N2: normal control cell line C14768. β-Actin was an internal loading control. Western blots showed protein Intensity of P1, P2, and P3 (HGPS cell culture HGADFN127, AG11498, and AG01972, respectively) is much stronger than that of control N1 (C10) and N2 (C14768).

Descrition of 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 background. Children with Progeria die of atherosclerosis (heart disease) at an average age of thirteen years (with a range of about 8 - 21 years).

http://www.progeriaresearch.org/about_progeria.html

pictures, book cover, and quote about the treatment



Wednesday, May 9, 2012

Cure for progeria

Although scientists are researching to find a cure,  as of right now there is no obvious or official treatment for progeria.  One thing they do know , however, is that there are a few drugs used for other diseases and anti-organ rejection, for people that had to have transplants, that reverse the aging symptom and reduce the progression of the progerin cell.  However, this is not a cure.  A lot more research and lab experiments have to be done for a cure to be discovered. 

Condensed progeria description

Progeria is a rare and fatal disease.  It is caused by a small mutation in a protien called Lamin A.  It is very unusual to have more than one child in the same family  affected by this disease.  When a child is affected by progeria, they are not mentally affected at all, but physically they experience drastic change in appearance.  They age four times as fast then a normal funtioning person, and ultimately look like an elderly person even at a young age. 

Progeria mutation