Forum for Science, Industry and Business

Sponsored by:     3M 
Search our Site:

 

Researchers develop mouse model for studying blinding diseases in humans

10.03.2005


Genetically engineered mice closely mimic what happens to people who have dry form of age-related macular degeneration



Researchers at the University of Utah have developed genetically engineered mice that closely mimic what happens to humans who suffer from the juvenile, or dry, form of age-related macular degeneration. The findings are reported in the March 4, 2005 Early Online Edition of the Proceedings of the National Academy of Sciences. The mice express a mutant ELOV4 gene that causes humans to develop from the juvenile form of macular degeneration known as Stargardt disease. The gene was discovered by the same team of University of Utah scientists in 2001. The mutation prevents retinal pigment epithelium cells (RPE) from disposing of cellular waste know as lipofuscin (including the fluorophore A2E).

This buildup of waste causes degeneration of the RPE cells in the central retina and results in progressive vision loss in both humans and mice with the mutation. "To date, these mice provide one of the best models to study both Stargardt disease and a dry form of age-related macular degeneration. Using these mice, it is now possible to test a variety of treatment strategies including cell transplantation, gene therapy and pharmaceuticals," said Kang Zhang, M.D., Ph.D., assistant professor of ophthalmology and visual sciences at University of Utah’s John A. Moran Eye Center and an investigator in the Program in Human Molecular Biology and Genetics at the University’s Eccles Institute of Human Genetics.


Zhang predicts human clinical trials for treatments of the disease could be available in the next three to four years. "By engineering how much of the gene mutation is expressed in the eyes of a mouse, we can create many different ways of the disease presentation and therefore many points to intervene in the disease process," he said.

Patients with Stargardt disease typically begin showing symptoms of vision loss between the ages of 10 and 20. While Zhang doesn’t expect new treatments to restore vision that has already been lost, he says it may be possible to stop further vision loss. Ultimately, he hopes to prevent vision loss before it occurs.

Age-related macular degeneration is the leading cause of blindness in Americans age 50 and older. There is currently no cure for the disease.

In addition to Zhang, the study included researchers from the University of Utah’s Department of Neurobiology and Anatomy, the University of California at San Diego, the Retina Foundation of the Southwest in Dallas, and Brigham Young University.

Kang Zhang, M.D., Ph.D | EurekAlert!
Further information:
http://www.hmbg.utah.edu
http://www.pnas.org

More articles from Life Sciences:

nachricht Meadows beat out shrubs when it comes to storing carbon
23.11.2017 | Norwegian University of Science and Technology

nachricht Migrating Cells: Folds in the cell membrane supply material for necessary blebs
23.11.2017 | Westfälische Wilhelms-Universität Münster

All articles from Life Sciences >>>

The most recent press releases about innovation >>>

Die letzten 5 Focus-News des innovations-reports im Überblick:

Im Focus: Frictional Heat Powers Hydrothermal Activity on Enceladus

Computer simulation shows how the icy moon heats water in a porous rock core

Heat from the friction of rocks caused by tidal forces could be the “engine” for the hydrothermal activity on Saturn's moon Enceladus. This presupposes that...

Im Focus: Nanoparticles help with malaria diagnosis – new rapid test in development

The WHO reports an estimated 429,000 malaria deaths each year. The disease mostly affects tropical and subtropical regions and in particular the African continent. The Fraunhofer Institute for Silicate Research ISC teamed up with the Fraunhofer Institute for Molecular Biology and Applied Ecology IME and the Institute of Tropical Medicine at the University of Tübingen for a new test method to detect malaria parasites in blood. The idea of the research project “NanoFRET” is to develop a highly sensitive and reliable rapid diagnostic test so that patient treatment can begin as early as possible.

Malaria is caused by parasites transmitted by mosquito bite. The most dangerous form of malaria is malaria tropica. Left untreated, it is fatal in most cases....

Im Focus: A “cosmic snake” reveals the structure of remote galaxies

The formation of stars in distant galaxies is still largely unexplored. For the first time, astron-omers at the University of Geneva have now been able to closely observe a star system six billion light-years away. In doing so, they are confirming earlier simulations made by the University of Zurich. One special effect is made possible by the multiple reflections of images that run through the cosmos like a snake.

Today, astronomers have a pretty accurate idea of how stars were formed in the recent cosmic past. But do these laws also apply to older galaxies? For around a...

Im Focus: Visual intelligence is not the same as IQ

Just because someone is smart and well-motivated doesn't mean he or she can learn the visual skills needed to excel at tasks like matching fingerprints, interpreting medical X-rays, keeping track of aircraft on radar displays or forensic face matching.

That is the implication of a new study which shows for the first time that there is a broad range of differences in people's visual ability and that these...

Im Focus: Novel Nano-CT device creates high-resolution 3D-X-rays of tiny velvet worm legs

Computer Tomography (CT) is a standard procedure in hospitals, but so far, the technology has not been suitable for imaging extremely small objects. In PNAS, a team from the Technical University of Munich (TUM) describes a Nano-CT device that creates three-dimensional x-ray images at resolutions up to 100 nanometers. The first test application: Together with colleagues from the University of Kassel and Helmholtz-Zentrum Geesthacht the researchers analyzed the locomotory system of a velvet worm.

During a CT analysis, the object under investigation is x-rayed and a detector measures the respective amount of radiation absorbed from various angles....

All Focus news of the innovation-report >>>

Anzeige

Anzeige

Event News

Ecology Across Borders: International conference brings together 1,500 ecologists

15.11.2017 | Event News

Road into laboratory: Users discuss biaxial fatigue-testing for car and truck wheel

15.11.2017 | Event News

#Berlin5GWeek: The right network for Industry 4.0

30.10.2017 | Event News

 
Latest News

Underwater acoustic localization of marine mammals and vehicles

23.11.2017 | Information Technology

Enhancing the quantum sensing capabilities of diamond

23.11.2017 | Physics and Astronomy

Meadows beat out shrubs when it comes to storing carbon

23.11.2017 | Life Sciences

VideoLinks
B2B-VideoLinks
More VideoLinks >>>