Friday, May 2, 2014

HGPS and Remodelin

     

     At the University of Cambridge, scientists have found a chemical that can repair cells damaged by a disease called Hutchinson-Gilford Progeria Syndrome (HGPS) involving accelerated aging. This disease is extremely rare; only 150 people worldwide are known to have HGPS. This disease is a genetic mutation that is not inherited. People with this disease only live until their early teenage years. This disease causes cell nuclei to be misshapen with symptoms including aging, brittle bones, stroke, and heart attacks. The mutation occurs in a gene that makes the proteins Lamin A and Lamin C. Scientists discovered a molecule named Remodelin that improved the misshapen nuclei and restored their round shape and also improved cell health. Now cures for HGPS using the Remodelin are underway. These discoveries have helped in understanding normal aging as well.

                I have never heard of HGPS before and it sounds terrifying! It is exciting that people that suffer from this awful disease may soon have their full effective cure. Remodelin works differently from the drugs that are only semi-effective that currently exists, so HGPS sufferers will hopefully have another shot at a normal life! Find out more about HGPS here.     

2 comments:

  1. this does sound very terrifying. having heard of this discovery gives us humans an advantage on working to treatments and cures!

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  2. Wow, this disease sounds terrible! It's very lucky for those that are affected that there is research being done to help it though, because much of the time with diseases at such small numbers there is not much funding for research. I'm glad that they are getting close to finding help for those people though!

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