Featured Research

from universities, journals, and other organizations

RNA-binding Protein Key To One Form Of Muscular Dystrophy

Date:
October 13, 2007
Source:
Baylor College of Medicine
Summary:
Increased levels of a protein called CUGBP1 play an important role in the adult-onset form of muscular dystrophy called myotonic dystrophy type 1 according to a new article.

Increased levels of a protein called CUGBP1 play an important role in the adult-onset form of muscular dystrophy called myotonic dystrophy type 1, said Baylor College of Medicine researchers in a report that appeared in the journal Molecular Cell.

Related Articles


Myotonic dystrophy type 1 is one of a growing number of newly recognized RNA diseases. RNA (ribonucleic acid) or messenger RNA takes the "message" about which proteins to make from the DNA to the protein manufacturing apparatus in the cell's interior or cytoplasm.

Myotonic dystrophy type 1 is the most common adult-onset form of muscular dystrophy. It is associated with hundreds and even thousands of repeats of the nucleotides CTG within a gene called DM kinase protein gene or DMPK. [Cytosine (C), thymine (T), guanine (G) and adenine (A) are all nucleotides that make up DNA. C, G, A, and uracil (U) make up RNA.]

In myotonic dystrophy type 1, the levels of CUGBP1 go up in heart and skeletal muscle, two tissues affected in the disease, because the protein becomes phosphorylated or acquires a phosphate molecule, which causes it to remain in the cell longer than it normally would.

"Normally, during heart development, CUGBP1 regulates alternative splicing (the building of the RNA message that determines which proteins are made by the cell)," said Dr. Thomas A. Cooper, professor of pathology at BCM and senior author of the paper. "But CUGBP1 goes away during normal heart development. In adults, the levels of CUGBP1 should be low."

However, when the RNA nucleotide repeats are expressed, they stimulate a signal that is normally present in embryonic tissues and not in adults. Specifically, the protein kinase C pathway is reactivated resulting in the addition of the phosphate molecules to CUGBP1. With the addition of the phosphate molecules, CUGBP1 levels increase to those observed in the embryonic period.

"This affects alternative splicing," said Cooper. "It changes the splicing from an adult pattern to an embryonic pattern. Adult tissues express embryonic proteins, and that's why people get sick."

Another RNA-binding protein called muscleblind like 1 (MBNL1) is also involved in the disease. It binds genetic material in the nucleus of the cell, trapping the RNA there and preventing it from transporting the RNA messages into the cell's cytoplasm. MBNL also becomes trapped with the RNA rendering it unable to function.

"In addition to gaining a better understanding of what causes this disease, we have learned that both CUGBP1 and MBNL1 regulate splicing of many genes during normal muscle development," said Cooper.

Others who took parting this research include Drs. N. Muge Kuyumcu-Martinez and Guey-Shin Wang, both of BCM. Funding for this research came from the National Institutes of Health and the Muscular Dystrophy Association.


Story Source:

The above story is based on materials provided by Baylor College of Medicine. Note: Materials may be edited for content and length.


Cite This Page:

Baylor College of Medicine. "RNA-binding Protein Key To One Form Of Muscular Dystrophy." ScienceDaily. ScienceDaily, 13 October 2007. <www.sciencedaily.com/releases/2007/10/071011125348.htm>.
Baylor College of Medicine. (2007, October 13). RNA-binding Protein Key To One Form Of Muscular Dystrophy. ScienceDaily. Retrieved December 20, 2014 from www.sciencedaily.com/releases/2007/10/071011125348.htm
Baylor College of Medicine. "RNA-binding Protein Key To One Form Of Muscular Dystrophy." ScienceDaily. www.sciencedaily.com/releases/2007/10/071011125348.htm (accessed December 20, 2014).

Share This


More From ScienceDaily



More Health & Medicine News

Saturday, December 20, 2014

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

The Best Tips to Curb Holiday Carbs

The Best Tips to Curb Holiday Carbs

Buzz60 (Dec. 19, 2014) It's hard to resist those delicious but fattening carbs we all crave during the winter months, but there are some ways to stay satisfied without consuming the extra calories. Vanessa Freeman (@VanessaFreeTV) has the details. Video provided by Buzz60
Powered by NewsLook.com
Sierra Leone Bikers Spread the Message to Fight Ebola

Sierra Leone Bikers Spread the Message to Fight Ebola

AFP (Dec. 19, 2014) More than 100 motorcyclists hit the road to spread awareness messages about Ebola. Nearly 7,000 people have now died from the virus, almost all of them in west Africa, according to the World Health Organization. Video provided by AFP
Powered by NewsLook.com
Researchers Test Colombian Village With High Alzheimer's Rates

Researchers Test Colombian Village With High Alzheimer's Rates

AFP (Dec. 19, 2014) In Yarumal, a village in N. Colombia, Alzheimer's has ravaged a disproportionately large number of families. A genetic "curse" that may pave the way for research on how to treat the disease that claims a new victim every four seconds. Duration: 02:42 Video provided by AFP
Powered by NewsLook.com
The Best Protein-Filled Foods to Energize You for the New Year

The Best Protein-Filled Foods to Energize You for the New Year

Buzz60 (Dec. 19, 2014) The new year is coming and nothing will energize you more for 2015 than protein-filled foods. Fitness and nutrition expert John Basedow (@JohnBasedow) gives his favorite high protein foods that will help you build muscle, lose fat and have endless energy. Video provided by Buzz60
Powered by NewsLook.com

Search ScienceDaily

Number of stories in archives: 140,361

Find with keyword(s):
Enter a keyword or phrase to search ScienceDaily for related topics and research stories.

Save/Print:
Share:

Breaking News:

Strange & Offbeat Stories


Health & Medicine

Mind & Brain

Living & Well

In Other News

... from NewsDaily.com

Science News

Health News

Environment News

Technology News



Save/Print:
Share:

Free Subscriptions


Get the latest science news with ScienceDaily's free email newsletters, updated daily and weekly. Or view hourly updated newsfeeds in your RSS reader:

Get Social & Mobile


Keep up to date with the latest news from ScienceDaily via social networks and mobile apps:

Have Feedback?


Tell us what you think of ScienceDaily -- we welcome both positive and negative comments. Have any problems using the site? Questions?
Mobile: iPhone Android Web
Follow: Facebook Twitter Google+
Subscribe: RSS Feeds Email Newsletters
Latest Headlines Health & Medicine Mind & Brain Space & Time Matter & Energy Computers & Math Plants & Animals Earth & Climate Fossils & Ruins