Biglycan to the Rescue!!

on Thursday, March 31, 2011

Walking across campus, raising your hand in class, and even typing on a keyboard are all actions which we complete on a daily basis without much thought. However, each is quite complex on the cellular level and, among other processes, requires your muscles to contract. Luckily, such action is automatic, and we don't have to tell ourselves to release say, actylcholine at a neuromuscular junction every time we move a muscle. However, with this innate response we often tend to forget how lucky we are to perform such simple actions. Unfortunately, some individuals do not have this gift. Those diagnosed with Muscular Dystrophy have weak muscles due to insufficient proteins and are unable to complete such actions previously listed. Sadly there is no known treatment for this disease, until now....

An article published by Science Daily in December 2010 described a novel treatment which may save those diagnosed with Duchenne Muscular Dystrophy. Individuals with this disease are unable to produce dystrophin (a crucial protein which keeps muscle cells strong). Early symptoms, include by the age of eight they are unable to walk, and generally do not live past their 20's. The solutions to this protein deficiency is to supply the body with biglycan (another protein) which restores the key protein utrophin which strengthens muscles. Utrophin is usually only found in children. Although low levels are present in adults there is not a high enough concentration to prevent the adverse affects of Muscular Dystrophy. When biglycan is added to the patients bloodstream, utrophin is drawn out from muscle cell membranes. Once, activated by the presence of biglycan, the protein utrophin then helps rebuild and maintain cellular strength.

Although humans have not been tested, initial laboratory experiments revealed 50% less of the cells treated with biglycan were damaged from stress tests, indicating they were indeed stronger. The image depicts cells treated with biglycan (bottom) vs. the absence of biglycan (top). Hopefully this method will prove to be effective to humans and provide a solution to this disease.


Amy said...

How exciting! I wonder when this will be used in humans since the research processes take so long to come to market? The images of the results are very impressive. Interesting post!

Patrick Schnieders said...

Great post! I learned a lot more about how muscle protection and repair works without getting bored out of my skull.

I find it strange that Utrophin is not found in adults. I wonder why that is? I'll have to look into it.

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