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:7/22/2014)... China , 22 juli 2014 BGI ... BGI, ,s wereld grootste organisatie op het gebied van ... service ten behoeve van hele menselijke genoomsequentie via het ... In 2013 acquireerde BGI Complete Genomics, ... staat vanwege de in eigen beheer ontwikkelde sequentietechnologieën met ...
(Date:7/22/2014)... In chemical and petrochemical plants, ... sight. The rapid rise of TDL analyzers over ... as a core gas measurement technology, and their ... the gas analyzer technology of choice for many ... some processes have limited their application range. METTLER ...
(Date:7/22/2014)... Seattle, WA (PRWEB) July 22, 2014 Quorum ... a new service for large clinical trials called Quick ... and is designed to address the research industry’s need for ... The service is available for studies planning 50 or ... with a combined planned North American site count of 80 ...
(Date:7/22/2014)... Juli 2014 Qforma, ein führender Anbieter ... von Marcus Bergler zum General Manager ... europäischen kommerziellen Aktivitäten von Qforma bereits als Vice ... anschloss, war er in Positionen als VP Vertrieb ... Deutschland bei IMS Health und als Direktor bei ...
Breaking Biology Technology:BGI Tech introduceert service op gebied van hele menselijke exoomsequencing op geavanceerd Complete Genomics-platform 2METTLER TOLEDO Shows How Tunable Diode Lasers (TDLs) Can Be Installed Where No TDLs Have Gone Before 2Quorum Review IRB Launches New Quick Step™ Service for Large Clinical Trials 2Quorum Review IRB Launches New Quick Step™ Service for Large Clinical Trials 3Qforma gibt Schaffung des neuen Postens als General Manager in Europa bekannt 2
... Red Herring has announced,that ProGenTech Limited was a winner of the ... technology companies based in Asia. , ... Asia and identified the top 100 out of more than 1,000,closely ... "It is truly an honor ...
... the U.S. Patent and Trademark Office has ... for Traumatic Injury to the Nervous System”, ... University of Florida with the company as ... method of determining the presence of neural ...
... Inc.,(Nasdaq: BNVI ) will present findings on the ... Annual San Antonio Breast Cancer Symposium,being held at the Henry ... Bionovo,s poster presentation will ... BN107. As explained by Dr. Emma Shtivelman,Director of Cancer Research ...
Cached Biology Technology:Red Herring Awards ProGenTech Limited for the 2008 Red Herring Asia 100 2 Banyan Biomarkers Announces Issuance of U.S. Patent for Biomarkers for Traumatic Injury 2Bionovo to Present Recent Findings of BN107, its Anticancer Agent, at the 31st Annual San Antonio Breast Cancer Symposium 2
(Date:7/22/2014)... allergic to dust mites (and chances are you are), ... the University of Iowa have developed a vaccine that ... immune response. In animal tests, the nano-sized vaccine package ... to the allergens, according to the paper, published in ... Journal . One big reason why it works, ...
(Date:7/22/2014)... Health Policy and Economics have linked tighter Medicaid ... for schizophrenic individuals. , The study comes amid ... actually save money, when other costs are taken ... extra approval step before tests or treatments can ... prior authorization is intended to encourage physicians ...
(Date:7/22/2014)... be on the horizon. Bacteria that produce a ... insulin resistance and other adverse effects of a ... discovered., "Of course it,s hard to speculate from ... Ph.D., assistant professor of Pharmacology. "But essentially we,ve ... in mice, even though they,re eating a high-fat ...
Breaking Biology News(10 mins):Researchers create vaccine for dust-mite allergies 2Are state Medicaid policies sentencing people with mental illnesses to prison? 2Vanderbilt study shows therapeutic bacteria prevent obesity in mice 2
... lice treatments such as the pyrethroids cypermethrin and deltamethrin, ... increased from 2008 to 2009, although use of emamectine ... again due to resistance problems, e.g. azametiphos, diflubenzuron and ... 1997, was also used again in 2009, mainly under ...
... The National Foundation for Cancer Research announced today ... the Department of Molecular and Experimental Medicine at The ... of the 5th Annual Szent-Gyrgyi Prize for Progress in ... on a humble chicken virus in the early 1960s, ...
... older than previously thought, says a new analysis of the ... plants, or angiosperms, first arose 140 to 190 million years ... Proceedings of the National Academy of Sciences pushes back ... 25 to 75 million years earlier than either the fossil ...
Cached Biology News:5th Annual Szent-Györgyi Prize for Progress in Cancer Research awarded 25th Annual Szent-Györgyi Prize for Progress in Cancer Research awarded 3Molecular study could push back angiosperm origins 2Molecular study could push back angiosperm origins 3
... cDNA Library Panels were designed for full-length ... of PCRs to identify the desired cDNA ... in a 96-well "Master Plate," where each ... Having identified the positive well(s) by gel ...
... Library Panels were designed for full-length gene ... PCRs to identify the desired cDNA clone. ... a 96-well "Master Plate," where each well ... identified the positive well(s) by gel electrophoresis ...
... Technologys R.A.P.I.D. System (Ruggedized Advanced Pathogen ... PCR thermocycler designed for field hospitals, ... In addition to its ruggedness, it ... and specify as the LightCycler instrument. ...
Application: For use as a carrier in nucleic acid purification and precipitation. Physical form: Solution in 10 mM Tris HCl, pH 7.4, 1 mM EDTA Concentration: 9-11 mg/mL Physical Form: buffered aqu...
Biology Products: