Featured Research

from universities, journals, and other organizations

Of Mice, Rabbits, And Men: New Rabbit Model Of Sudden Cardiac Death Provides Insight Into The Human Disease

Date:
May 11, 2008
Source:
Journal of Clinical Investigation
Summary:
Individuals with long QT syndrome are at increased risk of sudden death due to irregular heartbeats (also known as a cardiac arrhythmias). Although mutations in several genes have been shown to cause the disease, the most commonly affected genes are KCNQ1 and KCNH2.

Individuals with long QT syndrome (LQTS) are at increased risk of sudden death due to irregular heartbeats (also known as a cardiac arrhythmias). Although mutations in several genes have been shown to cause the disease, the most commonly affected genes are KCNQ1 and KCNH2.

New insight into the mechanisms by which these mutations might produce an irregular heartbeat in humans has been provided by Gideon Koren and colleagues, at Brown University, Providence, who have generated two rabbit models of LQTS.

Although mouse models of LQTS have enhanced our understanding of many aspects of the disease, there are important differences between mice and humans that limit the applicability of some studies to individuals with LQTS.

Many of these differences are not found in rabbits, the authors therefore generated rabbits expressing either KCNQ1 or KCNH2 mutants that generate proteins with the same functional defect as found in individuals with LQTS. The heart defects observed in the rabbits mirrored those observed in individuals with LQTS and more than 50% of the rabbits carrying the KCNH2 mutant died suddenly due to irregular heartbeats in their first year of life.

The proteins generated by the KCNQ1 and KCNH2 mutants prevented proteins generated by the corresponding nonmutant form of the gene from working and, importantly, no compensation for the loss of function of either protein was observed. As compensation for the loss of function of the proteins generated by the KCNQ1 and KCNH2 mutants is observed in mouse models of LQTS these data provide important insight into the mechanisms underlying the disease.


Story Source:

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


Journal Reference:

  1. Mechanisms of cardiac arrhythmias and sudden death in transgenic rabbits with long QT syndrome. Journal Of Clinical Investigation. May 8, 2008.

Cite This Page:

Journal of Clinical Investigation. "Of Mice, Rabbits, And Men: New Rabbit Model Of Sudden Cardiac Death Provides Insight Into The Human Disease." ScienceDaily. ScienceDaily, 11 May 2008. <www.sciencedaily.com/releases/2008/05/080508174839.htm>.
Journal of Clinical Investigation. (2008, May 11). Of Mice, Rabbits, And Men: New Rabbit Model Of Sudden Cardiac Death Provides Insight Into The Human Disease. ScienceDaily. Retrieved August 22, 2014 from www.sciencedaily.com/releases/2008/05/080508174839.htm
Journal of Clinical Investigation. "Of Mice, Rabbits, And Men: New Rabbit Model Of Sudden Cardiac Death Provides Insight Into The Human Disease." ScienceDaily. www.sciencedaily.com/releases/2008/05/080508174839.htm (accessed August 22, 2014).

Share This




More Health & Medicine News

Friday, August 22, 2014

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

Drug Used To Treat 'Ebola's Cousin' Shows Promise

Drug Used To Treat 'Ebola's Cousin' Shows Promise

Newsy (Aug. 21, 2014) An experimental drug used to treat Marburg virus in rhesus monkeys could give new insight into a similar treatment for Ebola. Video provided by Newsy
Powered by NewsLook.com
Two US Ebola Patients Leave Hospital Free of the Disease

Two US Ebola Patients Leave Hospital Free of the Disease

AFP (Aug. 21, 2014) Two American missionaries who were sickened with Ebola while working in Liberia and were treated with an experimental drug are doing better and have left the hospital, doctors say on August 21, 2014. Duration: 01:05 Video provided by AFP
Powered by NewsLook.com
Cadavers, a Teen, and a Medical School Dream

Cadavers, a Teen, and a Medical School Dream

AP (Aug. 21, 2014) Contains graphic content. He's only 17. But Johntrell Bowles has wanted to be a doctor from a young age, despite the odds against him. He was recently the youngest participant in a cadaver program at the Indiana University NW medical school. (Aug. 21) Video provided by AP
Powered by NewsLook.com
American Ebola Patients Released: What Cured Them?

American Ebola Patients Released: What Cured Them?

Newsy (Aug. 21, 2014) It's unclear whether the American Ebola patients' recoveries can be attributed to an experimental drug or early detection and good medical care. Video provided by Newsy
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