Navigation Links
A new mouse model provides insight into genetic neurological disorders
Date:5/25/2009

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), dmm.biologists.org> mice with mutations in the GARS gene have some of the same symptoms as CMT and HMN patients. Their hope is that this mouse can be used to study what causes these diseases and how it might effectively be treated. Mice with defects in some of this protein have problem with grip strength and motor skills while symptoms are more pronounced in animals that carry the mutation in all of their protein copies. This report is the first documentation of successful breeding of animals with this mutation, giving researchers access to new materials to understand how this gene influences human neuronal diseases.

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.


'/>"/>

Contact: Kristy Kain
kristy.kain@vanderbilt.edu
The Company of Biologists
Source:Eurekalert

Related biology news :

1. A computer for your mouse!
2. Immune cells promote blood vessel formation in mouse endometriosis
3. Cancer-resistant mouse discovered
4. Looking through the eyes of a mouse, scientists monitor circulating cells in its bloodstream
5. New chimeric mouse model for human liver diseases, drug testing
6. CSHL scientists identify and repress breast cancer stem cells in mouse tissue
7. Dissecting the genetic components of adaptation of E. coli to the mouse gut
8. Book is the first high-resolution digital mouse brain atlas designed for Web applications
9. Scientists successfully treat new mouse model of inflammatory bowel disease
10. Negligent, attentive mouse mothers show biological differences
11. Mouse study: When it comes to living longer, its better to go hungry than go running
Post Your Comments:
*Name:
*Comment:
*Email:
(Date:2/6/2017)... 6, 2017 According to Acuity Market ... border authorities to continue to embrace biometric and ... 2143 Automated Border Control (ABC) eGates and 1436 ... more than 163 ports of entry across the ... achieving a combined CAGR of 37%. APC Kiosks ...
(Date:2/2/2017)... 2017  Central to its deep commitment to ... The Japan Prize Foundation today announced the laureates ... the envelope in their respective fields of Life ... are being recognized with the 2017 Japan Prize ... contribute to the advancement of science and technology, ...
(Date:1/26/2017)... -- Acuity Market Intelligence today released the 2017 "Ten ... characterizes 2017 as a "breakout" year for biometrics ... new understanding of the potential benefits these technologies ... are often perceived as threats to privacy and ... Acuity Market intelligence. "However, taken together these technologies ...
Breaking Biology News(10 mins):
(Date:2/24/2017)... ... February 24, 2017 , ... FireflySci, Inc is an explosive small business ... had the goal of bringing their powerful cuvette and spectrophotometer calibration to ... that FireflySci is going on as they add yet another mark on the global ...
(Date:2/23/2017)... RAFAEL, Calif., Feb. 23, 2017 ... U.S. dollars, except per share data, unaudited)Three Months Ended ... ChangeTotal BioMarin Revenue $   ...   22832%$ 1,117$   89026%Aldurazyme Net Product Revenue ... 906538%34823946%Naglazyme Net Product Revenue  ...
(Date:2/23/2017)... Feb. 23, 2017  Seattle,s upscale Capitol Hill neighborhood, with its ... place for a head lice treatment salon to set up ... Tuscan restaurant and a French bistro on E Madison Ave, ... aren,t just any old lice clinic, we pride ourselves on ... and release some of the stigma associated with lice. Everyone ...
(Date:2/23/2017)... ... February 23, 2017 , ... ... insulin, cortisol, CRP, adiponectin, uric acid, and/or other biomarkers or SNPs of interest) ... from Salimetrics’ SalivaLab , the relationship between insulin and other relevant biomarkers ...
Breaking Biology Technology: