Featured Research

from universities, journals, and other organizations

Faulty Cell Membrane Repair Causes Heart Disease

Date:
July 6, 2007
Source:
University of Iowa
Summary:
During vigorous exercise, heart muscle cells take a beating. In fact, some of those cells rupture and without an efficient repair process those cells would die and cause heart damage (cardiomyopathy). University of Iowa researchers have discovered a specific repair mechanism in heart muscle and identified a protein called dysferlin that is critical for resealing heart muscle cell membranes.

During vigorous exercise, heart muscle cells take a beating. In fact, some of those cells rupture, and if not for a repair process capable of resealing cell membranes, those cells would die and cause heart damage (cardiomyopathy).

Related Articles


Researchers at the University of Iowa Roy J. and Lucille A. Carver College of Medicine have discovered a specific repair mechanism in heart muscle and identified a protein called dysferlin that is critical for resealing heart muscle cell membranes.

The study, led by UI researcher and Howard Hughes Medical Institute investigator Kevin Campbell, Ph.D., also shows that loss of dysferlin causes cardiomyopathy in mice. Furthermore, heart damage in these mice is exaggerated by vigorous exercise or by inherent muscle weakness caused by a muscular dystrophy defect. The results are published in the July 1 issue of the Journal of Clinical Investigation.

Active tissues, like a beating heart or contracting muscle, need mechanisms to repair the inevitable cell membrane tears caused by physical stress and strain. In 2003, Campbell and his colleagues identified dysferlin as a key protein in this vital repair mechanism in skeletal muscle. In humans, dysferlin deficiency -- which leads to faulty muscle membrane repair -- causes three types of muscular dystrophy.

The new study expands knowledge of dysferlin function, showing that dysferlin-mediated membrane repair is also important in heart muscle cells and suggests that inadequate membrane repair can also lead to cardiomyopathy.

"If we could boost this repair mechanism, it might be possible to slow cardiac and skeletal muscle damage in muscular dystrophy patients," said Campbell who also holds the Roy J. Carver Biomedical Research Chair in Molecular Physiology and is head of the department and a UI professor of neurology.

The UI team initially found that young mice that lacked dysferlin showed no heart damage, which is consistent with what is seen in humans with dysferlin mutations. However, a case study describing late-onset cardiomyopathy in a Japanese patient with a dysferlin deficiency prompted the UI team to look at the mice as they aged.

They found that the mice started to develop cardiomyopathy at about one year of age (middle aged for a mouse). The team also found that exercise exaggerated the stress-induced injury in these mice, suggesting that inadequate membrane repair led to cardiomyopathy.

The research team also bred mice that lacked both dysferlin and the protein dystrophin, which is missing in patients with Duchenne muscular dystrophy. These "double knockout" mice had early onset cardiomyopathy, which was much more severe than in mice with either of the single mutations. The results suggest that dysferlin might provide some protection against heart damage in Duchenne patients, at least at a young age, by delaying the onset of cardiomyopathy.

"We hope these findings will stimulate clinicians to look at the cardiac health of muscular dystrophy patients and the overall muscle health of patients with cardiomyopathy," Campbell said.

In addition to Campbell, the research team included Renzhi Han, Ph.D., a research scientist in Campbell's lab and first author of the study, and Robert Weiss, M.D., UI professor of internal medicine. Dimple Bansal, Ph.D., and Viviane Muniz in Campbell's lab, and Paul McNeil and Katsuya Miyake, researchers at the Medical College of Georgia in Augusta, Ga., were also part of the team.

The study was funded in part by a National Institutes of Health grant for the Senator Paul D. Wellstone Muscular Dystrophy Cooperative Research Center at the UI.


Story Source:

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


Cite This Page:

University of Iowa. "Faulty Cell Membrane Repair Causes Heart Disease." ScienceDaily. ScienceDaily, 6 July 2007. <www.sciencedaily.com/releases/2007/07/070703171935.htm>.
University of Iowa. (2007, July 6). Faulty Cell Membrane Repair Causes Heart Disease. ScienceDaily. Retrieved December 20, 2014 from www.sciencedaily.com/releases/2007/07/070703171935.htm
University of Iowa. "Faulty Cell Membrane Repair Causes Heart Disease." ScienceDaily. www.sciencedaily.com/releases/2007/07/070703171935.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
Double-Amputee Becomes First To Move Two Prosthetic Arms With His Mind

Double-Amputee Becomes First To Move Two Prosthetic Arms With His Mind

Buzz60 (Dec. 19, 2014) A double-amputee makes history by becoming the first person to wear and operate two prosthetic arms using only his mind. Jen Markham has the story. 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