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 Newly designed molecule binds nitrogen
23.02.2018 | Julius-Maximilians-Universität Würzburg

nachricht Atomic Design by Water
23.02.2018 | Max-Planck-Institut für Eisenforschung GmbH

All articles from Life Sciences >>>

The most recent press releases about innovation >>>

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

Im Focus: Attoseconds break into atomic interior

A newly developed laser technology has enabled physicists in the Laboratory for Attosecond Physics (jointly run by LMU Munich and the Max Planck Institute of Quantum Optics) to generate attosecond bursts of high-energy photons of unprecedented intensity. This has made it possible to observe the interaction of multiple photons in a single such pulse with electrons in the inner orbital shell of an atom.

In order to observe the ultrafast electron motion in the inner shells of atoms with short light pulses, the pulses must not only be ultrashort, but very...

Im Focus: Good vibrations feel the force

A group of researchers led by Andrea Cavalleri at the Max Planck Institute for Structure and Dynamics of Matter (MPSD) in Hamburg has demonstrated a new method enabling precise measurements of the interatomic forces that hold crystalline solids together. The paper Probing the Interatomic Potential of Solids by Strong-Field Nonlinear Phononics, published online in Nature, explains how a terahertz-frequency laser pulse can drive very large deformations of the crystal.

By measuring the highly unusual atomic trajectories under extreme electromagnetic transients, the MPSD group could reconstruct how rigid the atomic bonds are...

Im Focus: Developing reliable quantum computers

International research team makes important step on the path to solving certification problems

Quantum computers may one day solve algorithmic problems which even the biggest supercomputers today can’t manage. But how do you test a quantum computer to...

Im Focus: In best circles: First integrated circuit from self-assembled polymer

For the first time, a team of researchers at the Max-Planck Institute (MPI) for Polymer Research in Mainz, Germany, has succeeded in making an integrated circuit (IC) from just a monolayer of a semiconducting polymer via a bottom-up, self-assembly approach.

In the self-assembly process, the semiconducting polymer arranges itself into an ordered monolayer in a transistor. The transistors are binary switches used...

Im Focus: Demonstration of a single molecule piezoelectric effect

Breakthrough provides a new concept of the design of molecular motors, sensors and electricity generators at nanoscale

Researchers from the Institute of Organic Chemistry and Biochemistry of the CAS (IOCB Prague), Institute of Physics of the CAS (IP CAS) and Palacký University...

All Focus news of the innovation-report >>>

Anzeige

Anzeige

VideoLinks
Industry & Economy
Event News

2nd International Conference on High Temperature Shape Memory Alloys (HTSMAs)

15.02.2018 | Event News

Aachen DC Grid Summit 2018

13.02.2018 | Event News

How Global Climate Policy Can Learn from the Energy Transition

12.02.2018 | Event News

 
Latest News

Basque researchers turn light upside down

23.02.2018 | Physics and Astronomy

Finnish research group discovers a new immune system regulator

23.02.2018 | Health and Medicine

Attoseconds break into atomic interior

23.02.2018 | Physics and Astronomy

VideoLinks
Science & Research
Overview of more VideoLinks >>>