Featured Research

from universities, journals, and other organizations

Mouse With Myotonic Dystrophy Type 1 Finds RNA Binding Proteins At Heart Of Problem

Date:
September 9, 2007
Source:
Baylor College of Medicine
Summary:
A new mouse model for myotonic dystrophy -- the most common form of adult-onset muscular dystrophy -- helped researchers show that levels of CUGBP1, a protein that binds and controls the activity of the genetic material RNA, increase early in affected cells of the animals with the disease. This means CUGBP1 plays a key role in the disorder.

A new mouse model for myotonic dystrophy -- the most common form of adult-onset muscular dystrophy -- helped Baylor College of Medicine researchers show that levels of CUGBP1, a protein that binds and controls the activity of the genetic material RNA, increase early in affected cells of the animals with the disease. This means CUGBP1 plays a key role in the disorder.

"We wanted to find out if this is a primary event associated with the disorder or if it is a secondary response to tissue injury," said Dr. Thomas A. Cooper, professor of pathology at BCM and senior author of the report that appeared recently in the Journal of Clinical Investigation.

Myotonic dystrophy type 1 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 the mouse that Cooper and his colleagues specially bred, the repeats in the gene can be turned on in heart, skeletal muscle and brain tissue at any age.

The researchers found that within three hours of turning on the repeats, another RNA-binding protein called muscleblind like (MBNL) began to bind the genetic material in the nucleus of the cell. That mean the RNA was trapped in the nucleus and unable to take the genetic message about which proteins to make to the protein manufacturing areas in the cytoplasm of the cell.

Within six hours, levels of CUGBP1 begin to increase. The increased in CUGBP1 then alters how a number of other genes are regulated. At that point, the cascade of events that affect the heart starts.

"The heart doesn't even 'know' that it is sick yet," said Cooper. This finding shows that the increase levels of CUGBP1 is an early event and plays an important role in the development of the disease.

Others who took part in this research include Drs. Guey-Shin Wang, Debra L. Kearney, Mariella De Biasi and George Taffet, all 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. "Mouse With Myotonic Dystrophy Type 1 Finds RNA Binding Proteins At Heart Of Problem." ScienceDaily. ScienceDaily, 9 September 2007. <www.sciencedaily.com/releases/2007/09/070905172004.htm>.
Baylor College of Medicine. (2007, September 9). Mouse With Myotonic Dystrophy Type 1 Finds RNA Binding Proteins At Heart Of Problem. ScienceDaily. Retrieved August 21, 2014 from www.sciencedaily.com/releases/2007/09/070905172004.htm
Baylor College of Medicine. "Mouse With Myotonic Dystrophy Type 1 Finds RNA Binding Proteins At Heart Of Problem." ScienceDaily. www.sciencedaily.com/releases/2007/09/070905172004.htm (accessed August 21, 2014).

Share This




More Health & Medicine News

Thursday, August 21, 2014

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

Possible Ebola Patient in Isolation at California Hospital

Possible Ebola Patient in Isolation at California Hospital

Reuters - US Online Video (Aug. 20, 2014) — A patient who may have been exposed to the Ebola virus is in isolation at the Kaiser Permanente South Sacramento Medical Center. Linda So reports. Video provided by Reuters
Powered by NewsLook.com
Raw: World's Oldest Man Lives in Japan

Raw: World's Oldest Man Lives in Japan

AP (Aug. 20, 2014) — A 111-year-old Japanese was certified as the world's oldest man by Guinness World Records on Wednesday. Sakari Momoi, a native of Fukushima in northern Japan, was given a certificate at a hospital in Tokyo. (Aug. 20) Video provided by AP
Powered by NewsLook.com
Do More Wedding Guests Make A Happier Marriage?

Do More Wedding Guests Make A Happier Marriage?

Newsy (Aug. 20, 2014) — A new study found couples who had at least 150 guests at their weddings were more likely to report being happy in their marriages. Video provided by Newsy
Powered by NewsLook.com
Ebola-Hit Sierra Leone's Freetown a City on Edge

Ebola-Hit Sierra Leone's Freetown a City on Edge

AFP (Aug. 19, 2014) — Residents of Sierra Leone's capital voice their fears as the Ebola virus sweeps through west Africa. Duration: 00:56 Video provided by AFP
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:
from the past week

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