Featured Research

from universities, journals, and other organizations

New Mouse Model Provides Insight Into Genetic Neurological Disorders

Date:
May 27, 2009
Source:
The Company of Biologists
Summary:
Neurosensory diseases are difficult to model in mice because their symptoms are complex and diverse. The genetic causes identified are often lethal when transferred to a mouse. The lack of animal models slows progress in understanding and treating the diseases. By strategically altering a protein-making molecule, a mouse was made to help understand nervous system diseases that impair feeling and cause paralysis of the arms and legs in humans.

Neurosensory diseases are difficult to model in mice because their symptoms are complex and diverse. The genetic causes identified are often lethal when transferred to a mouse. The lack of animal models slows progress in understanding and treating the diseases. By strategically altering a protein-making molecule, a mouse was made to help understand nervous system diseases that impair feeling and cause paralysis of the arms and legs in humans.

Scientists have created a mouse to help understand human neuronal diseases that impair a patient's ability to feel and to move their arms and legs. By strategically altering a protein-making molecule, a mouse was made with symptoms similar to the nervous system diseases, Charcot-Marie-Tooth (CMT) and hereditary motor neuropathy (HMN). In CMT and HMN, neurons that signal and maintain muscle cells become defective, which causes weakening and loss of muscle that is significant enough in some cases to lead to death. The symptoms become progressively worse over time and no effective treatments or cures exist for these diseases. Researchers came together from the University College London (UCL), the Medical Research Centre (MRC) Harwell, the University of Oxford, and the University of London in England, Vrije University in The Netherlands and Jackson Laboratories in the US to make a genetic change in mice that has been associated with CMT and HMN diseases in people.

Neurosensory diseases are difficult to model in mice because they involve symptoms that are complex and diverse. These diseases are passed from parents to their children but the genetic causes identified are often lethal when transferred to a mouse. The lack of animal models slows progress in understanding and treating the diseases.

The researchers made a mutation in a protein, which is part of the protein building machinery, called glycyl-tRNA synthetase (GARS). As described in their study in Disease Models & Mechanisms (DMM),

When the researchers made the same mutation in two different breeds of mice it caused two distinguishable sets of symptoms, demonstrating that the genetic background influences the effects of the GARS gene mutation. This variability in the mouse disease symptoms is also seen in humans, and may help shed light on how CMT and HMN differently affect individual patients' symptoms.

The report titled "An ENU-induced mutation in mouse glycyl tRNA synthetase (Gars) causes peripheral sensory and motor phenotypes creating a model of Charcot-Marie-Tooth type 2D peripheral neuropathy" was written by Francesca Achilli, Virginie Bros Facer, Hazel Williams, Gareth Banks, Mona AlQatari, Ruth Chia, Michael Groves, Sebastian Brandner, Martin Koltzenburg, Linda Greensmith, and Elizabeth M.C. Fisher at the University College London (UCL), Valter Tucci, Rachel Kendall and Patrick Nolan at the Medical Research Centre (MRC) Harwell, Carole Nickols and Joanne Martin at Queen Mary University of London, Kevin Seburn and Robert Burgess at Jackson Laboratories, Muhammed Cader and Kevin Talbot at the University of Oxford, and Jan van Minnen at Vrije University. The study is published in the June/July issue of the new research journal, Disease Models & Mechanisms (DMM), published by The Company of Biologists, a non-profit based in Cambridge, UK.


Story Source:

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


Journal Reference:

  1. Achilli et al. An ENU-induced mutation in mouse glycyl tRNA synthetase (Gars) causes peripheral sensory and motor phenotypes creating a model of Charcot-Marie-Tooth type 2D peripheral neuropathy. Disease Models & Mechanisms, 2009; DOI: 10.1242/dmm.002527

Cite This Page:

The Company of Biologists. "New Mouse Model Provides Insight Into Genetic Neurological Disorders." ScienceDaily. ScienceDaily, 27 May 2009. <www.sciencedaily.com/releases/2009/05/090526093934.htm>.
The Company of Biologists. (2009, May 27). New Mouse Model Provides Insight Into Genetic Neurological Disorders. ScienceDaily. Retrieved July 29, 2014 from www.sciencedaily.com/releases/2009/05/090526093934.htm
The Company of Biologists. "New Mouse Model Provides Insight Into Genetic Neurological Disorders." ScienceDaily. www.sciencedaily.com/releases/2009/05/090526093934.htm (accessed July 29, 2014).

Share This




More Health & Medicine News

Tuesday, July 29, 2014

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

Deadly Ebola Virus Threatens West Africa

Deadly Ebola Virus Threatens West Africa

AP (July 28, 2014) West African nations and international health organizations are working to contain the largest Ebola outbreak in history. It's one of the deadliest diseases known to man, but the CDC says it's unlikely to spread in the U.S. (July 28) Video provided by AP
Powered by NewsLook.com
$15B Deal on Vets' Health Care Reached

$15B Deal on Vets' Health Care Reached

AP (July 28, 2014) A bipartisan deal to improve veterans health care would authorize at least $15 billion in emergency spending to fix a veterans program scandalized by long patient wait times and falsified records. (July 28) Video provided by AP
Powered by NewsLook.com
Two Americans Contract Ebola in Liberia

Two Americans Contract Ebola in Liberia

Reuters - US Online Video (July 28, 2014) Two American aid workers in Liberia test positive for Ebola while working to combat the deadliest outbreak of the virus ever. Linda So reports. Video provided by Reuters
Powered by NewsLook.com
Traditional African Dishes Teach Healthy Eating

Traditional African Dishes Teach Healthy Eating

AP (July 28, 2014) Classes are being offered nationwide to encourage African Americans to learn about cooking fresh foods based on traditional African cuisine. The program is trying to combat obesity, heart disease and other ailments often linked to diet. (July 28) Video provided by AP
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