What a coincidence!

Just as I finished writing the post about modeling Progeria disease with stem cells, I came across this tweet from New Scientist:

The children who grow old before they grow up>> Could they hold key to longer lives? 

 

My take on this, is that Progeria is caused by the presence of Progerin, a mutated form a LMNA. So unless old people get this mutation and accumulate high levels of Progerin protein, it is a tough call to say that we can deduce conclusive information about the normal process of ageing from studying Progeria Syndrome.

Disease in a plate

All thanks to Dr Shinya Yamanaka, we are now able to make patient-specific induced pluripotent stem cells (iPSCs). If you are a stem cell virgin, please refer to my FAQs about stem cells page. 

Diagram from http://www.rndsystems.com.

For example, let’s look at a rare congenital disease, Hutchinson Gilford Progeria syndrome. Progeria syndrome causes premature ageing in children.

Progeria patient. Image from http://en.wikipedia.org/wiki/Progeria

The wrinkled facial features, wasted bodies, loss of hair, and stunted growth make this heart-wrenching disease familiar to the general masses.


Movies such as “Curious Case of Benjamin Button” and “Jack” were said to have been inspired by this disease. Progeria patients generally die in their early teens due to heart attack or stroke.

There is currently no treatment for Progeria syndrome.

In the above study, patient's skin cells were reprogrammed into iPSCs. These Progeria iPSCs were made into different cell types of the body to study how the disease emerges.


By making a "Progeria-patient-on-a-plate", they discovered which cell types were afflicted in these patients, and how. Such studies could be further used to test for drugs that treat these cell types!


Here's a cartoon to summarize the work flow for using stem cells for disease modeling.

Diagram modified from Colman and Dreesen Cell Stem Cell 2009.