Navigation Links
A Long-Awaited Mouse Offers a New Animal Model for FSH Muscular Dystrophy
Date:4/4/2013

definitive mouse model based on a genetic unit called D4Z4. Normally, people have ten or more of these units, repeated one after the other near the tip of chromosome 4. The majority of FSHD patients, in contrast, have fewer than ten.

The D4Z4 unit contains a gene called DUX4, which is toxic to muscle cells. In a series of landmark studies that unfolded over the past several years, an international team discovered that DUX4 is normally only expressed during embryonic development and in the male germline (in stem cells that give rise to sperm). DUX4 is not supposed to be active in other tissues, and the D4Z4 units act like bricks in a firewall, preventing the information in the DUX4 gene from getting out.

In FSHD patients, however, the reduced number of D4Z4 weakens the firewall, allowing DUX4 to be abnormally expressed in adult muscle in a quite remarkable pattern; only a small subset of muscle cell nuclei expresses abundant levels of DUX4. The result is devastating, as skeletal muscles degenerate, typically in the face (facio-), shoulder blades (scapula-) and upper arms (humeral), the anatomical areas initially affected that gave rise to the name of this dystrophy. The weakened muscles make it difficult for patients to blink or smile, or raise their arms overhead. FSHD can also affect leg and hip muscles, leading to falls, broken bones and dependence on a scooter or wheelchair. Some patients endure hearing loss and/or abnormalities of blood vessels in the back of the eye.

Around 500,000 people have FSHD worldwide. It is among the three most common muscular dystrophies, and between one and two percent of the general population carries a genetic risk factor linked to FSHD.

The newly published mouse model contains 2.5 copies of the D4Z4 unit, a truncated number comparable to that seen in human FSHD patients. A second line of mice was created with 12.5 D4Z4 units, corresponding to an unaffected person. Both mouse models had high levels
'/>"/>

Source: PRWeb
Copyright©2012 Vocus, Inc.
All rights reserved

Page: 1 2 3 4 5

Related biology technology :

1. University of Colorado Cancer Center Study Shows that Bitter Melon Juice Prevents Pancreatic Cancer in Mouse Models
2. The Veterinary Bioscience Institute Announces a Mouse Surgery Webinar Series
3. Kymab Announces Launch of Kymouse HK
4. SPX-106T Arrests Development of Atherosclerotic Plaques in Mouse Model of Cardiovascular Disease
5. A mouse model brings new perspectives on Lafora disease
6. Neighborhood Smiles of Green Bay Offers a New Approach to the Cavity Epidemic
7. Bed Bugs Get Family Throwing Nearly All Their Possessions, My Cleaning Offers Help With Complimentary Samples of Non-Pesticide Bed Bug Bully
8. Ag-Biotech Offers Male Sterility Locus Marker Services for Breeding Onions
9. Green Rust Remover Offers Complimentary Samples to Rust Contractors, My Cleaning Products Affirms It Could Cut Expenses Up to 50 Percent
10. Life Technologies Offers New Rapid DNA Platform
11. Three-Step Rust Removal With a Non-Invasive Rust Remover Published by My Cleaning Products, MCP Offers Sample of the Product
Post Your Comments:
*Name:
*Comment:
*Email:
(Date:8/21/2014)... An international research team including DESY scientists has observed ... The team reports in the journal Science ... lattices inside the nanodroplets. It is the first time ... in larger samples of what is known as superfluid ... exceeded our best expectations," says Andrey Vilesov of the ...
(Date:8/21/2014)... The College of New Rochelle announced ... the Mid-Hudson Region to be designated as a START-UP ... from qualified “high-technology” businesses that align with the College’s ... have been selected for the START-UP NY program and ... stimulate economic development in New York by growing business ...
(Date:8/21/2014)... 21, 2014  Four-dimensional (4-D) printing develops materials ... external stimuli such as changes in temperature. This ... effect in multiple industries.  In the near future, ... printing objects ranging from human organs to parts ... Logo - http://photos.prnewswire.com/prnh/20140820/138289 ...
(Date:8/21/2014)... On Wednesday of last week RENU 28, ... Signaling molecules, became available for purchase in Australia and New ... that RENU 28 works is, if you think about the ... rate of cellular renewal within your body. If you think ... rate of cellular renewal. What RENU 28 does is it ...
Breaking Biology Technology:Scientists observe quantum vortices in cold helium droplets 2The College of New Rochelle Becomes First Private College in Mid-Hudson Region to Earn START-UP NY Designation 2The College of New Rochelle Becomes First Private College in Mid-Hudson Region to Earn START-UP NY Designation 3Frost & Sullivan: 4-D Printing to Usher in Age of Low-Labor, Fast-Paced Product Manufacturing 2Frost & Sullivan: 4-D Printing to Usher in Age of Low-Labor, Fast-Paced Product Manufacturing 3Frost & Sullivan: 4-D Printing to Usher in Age of Low-Labor, Fast-Paced Product Manufacturing 4RENU 28 Skin Care Now Available in Australia and New Zealand 2
... VIEW, Calif., Feb. 27 Alexza,Pharmaceuticals, Inc. (Nasdaq: ... Simms,has joined the company as Senior Vice President, ... Vice President,Manufacturing Operations at Nektar Therapeutics. Mr. Simms ... Pharmaceuticals and Vice President,Device Development and Manufacturing from ...
... Calif., Feb. 27 /Xinhua-PRNewswire-FirstCall/ --,Kiwa Bio-Tech Products Group ... being considered as a supplier of bio-fertilizers,for the ... and hotels,during the 2008 Olympics. Kiwa also announced ... of the Company, would participate in an interview ...
... 27 DARA BioSciences(TM),(Nasdaq: DARA ) announced today ... of Directors. Mr. Cochrane was appointed to fill a ... Compensation Committees.,The appointment was effective February 21, 2008., ... Sr.,commented, "We are pleased that Haywood has joined us ...
Cached Biology Technology:Michael J. Simms Joins Alexza Pharmaceuticals as Senior Vice President, Operations and Manufacturing 2Michael J. Simms Joins Alexza Pharmaceuticals as Senior Vice President, Operations and Manufacturing 3Beijing 2008 Olympic Games Vegetable Supplier Considering Kiwa as a Supplier of Bio-Fertilizers & Kiwa Announces CEO Interview with WallSt.net 2Beijing 2008 Olympic Games Vegetable Supplier Considering Kiwa as a Supplier of Bio-Fertilizers & Kiwa Announces CEO Interview with WallSt.net 3DARA BioSciences, Inc. Announces Appointment of New Director to Board 2
(Date:8/22/2014)... . , Thanks ... in a way so as to align themselves symmetrically, ... University researchers are planning on looking into how PCFs ... being published today in the scientific journal Nature ... Everyone knows that at least from having boiled ...
(Date:8/22/2014)... reductase (DHCR24) is a multifunctional enzyme ... and has neuroprotective and cholesterol-synthesizing activities. ... caused by amyloid β deposition. Dr. ... University in China constructed two recombinant ... DHCR24 expression specifically in neuronal cells. ...
(Date:8/21/2014)... including researchers from the University of Georgia recently published the ... journal Science . Their discovery paves the way for ... farming and industry. , Canola is grown across much ... is increasingly cultivated in Georgia. Canola oil used for cooking ... and rich supply of omega-3 fatty acids, but the plant ...
Breaking Biology News(10 mins):Proteins: New class of materials discovered 2Canola genome sequence reveals evolutionary 'love triangle' 2
... at Duke's Pratt School of Engineering have imaged the ... showed that the probes could give surgeons a better ... performed through tiny "keyhole" incisions. , If the probes ... to more precise and safer endoscopic surgeries, said the ...
... soon be offered the opportunity to have their daughters ... major cause of cervical cancer, the 4th International Conference ... (Thursday 30 March). , Professor Henry Kitchener told the ... progress in developing vaccines against the human papillomavirus (HPV) ...
... ends of bones where they meet in a joint) ... lead to degenerative disease. Treatments and experimental approaches to ... currently there is no method to fully restore this ... of therapeutic proteins to the injured site is a ...
Cached Biology News:3D ultrasound device poised to advance minimally invasive surgery 23D ultrasound device poised to advance minimally invasive surgery 3Parents need to be educated about HPV vaccinations for daughters 2Parents need to be educated about HPV vaccinations for daughters 3Stem cells from muscles can repair cartilage 2Stem cells from muscles can repair cartilage 3
...
... DNA Purification Kit is designed for isolating DNA ... The protocol can be completed in less than ... obtain larger amounts of DNA. The purified DNA ... and Southern blotting. The MasterPure Kit was designed ...
Caspase Activity Detector, SR-VAD-FMK Kit , 100 tests Consult technical datasheet for details....
HistoScan AP Universal Goat Kit...
Biology Products: