Forum for Science, Industry and Business

Sponsored by:     3M 
Search our Site:

 

New mouse model will aid research on premature aging syndrome

15.05.2003


Researchers from the National Cancer Institute (NCI) have developed a mouse model of the premature aging syndrome known as Hutchinson-Gilford Progeria Syndrome (HGPS), according to a report appearing in the journal Nature. Researchers hope the mouse model will facilitate a better understanding of the fatal syndrome, as well as provide clues to the normal aging process.



Currently, there is no treatment for progeria, and children with the rare condition usually die of heart disease in their early teens. Although normal at birth, children with progeria begin to develop growth retardation, thinning skin, and fragile bones as young as 18 months.

"The similarities between mice with this particular mutation and patients with progeria are remarkable," said Colin Stewart, Ph.D., of NCI’s Center for Cancer Research, the senior investigator on the study. "Now that we’ve identified the critical gene and have an animal model that mimics progeria, we have powerful tools for studying both the aging process and this devastating disease."


The results of the animal study come less than a month after the announcement that scientists have discovered the gene responsible for progeria in children. Studies published in March in the journals Science and Nature described a single inaccuracy in the Lamin A (Lmna) gene that appears to account for the syndrome. The gene produces structural proteins known as lamins, which are found in the cell nucleus. In the new study, NCI researchers report that mice with a specific mutation in the same gene have symptoms remarkably consistent with those of progeria patients.

Although indistinguishable from their littermates at birth, mice with the Lmna mutation develop severe growth retardation early in life and die within five weeks, whereas normal mice generally live up to two years. Like progeria patients, mice in the study showed signs of premature aging.

In the mice, accelerated aging was most apparent in the skin, which thinned dramatically and lost hair. Researchers also observed reduced growth or degeneration of the heart and skeletal muscles in mutant mice. Similarly, the mice had either incomplete development of the skeleton or a premature loss of bone mass, also characteristic of children with progeria. Mice with the Lmna mutation shared many other symptoms with progeria patients, including a slight waddling gait, abnormal teeth, and incomplete sexual maturation.

The Lmna gene codes for A- and C-type lamins, components of the fibrous network lining the inside of the cell nucleus called the nuclear lamina. The mutant form of the gene associated with progeria symptoms causes disruption in the structure of the cell nucleus. Studying the relationship between progeria symptoms and this abnormality could help researchers understand the cellular processes associated with aging.

NCI Press Officer | EurekAlert!
Further information:
http://www.nci.nih.gov/
http://cancer.gov

More articles from Health and Medicine:

nachricht The genes are not to blame
20.07.2018 | Technische Universität München

nachricht Targeting headaches and tumors with nano-submarines
20.07.2018 | Universitätsmedizin der Johannes Gutenberg-Universität Mainz

All articles from Health and Medicine >>>

The most recent press releases about innovation >>>

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

Im Focus: Future electronic components to be printed like newspapers

A new manufacturing technique uses a process similar to newspaper printing to form smoother and more flexible metals for making ultrafast electronic devices.

The low-cost process, developed by Purdue University researchers, combines tools already used in industry for manufacturing metals on a large scale, but uses...

Im Focus: First evidence on the source of extragalactic particles

For the first time ever, scientists have determined the cosmic origin of highest-energy neutrinos. A research group led by IceCube scientist Elisa Resconi, spokesperson of the Collaborative Research Center SFB1258 at the Technical University of Munich (TUM), provides an important piece of evidence that the particles detected by the IceCube neutrino telescope at the South Pole originate from a galaxy four billion light-years away from Earth.

To rule out other origins with certainty, the team led by neutrino physicist Elisa Resconi from the Technical University of Munich and multi-wavelength...

Im Focus: Magnetic vortices: Two independent magnetic skyrmion phases discovered in a single material

For the first time a team of researchers have discovered two different phases of magnetic skyrmions in a single material. Physicists of the Technical Universities of Munich and Dresden and the University of Cologne can now better study and understand the properties of these magnetic structures, which are important for both basic research and applications.

Whirlpools are an everyday experience in a bath tub: When the water is drained a circular vortex is formed. Typically, such whirls are rather stable. Similar...

Im Focus: Breaking the bond: To take part or not?

Physicists working with Roland Wester at the University of Innsbruck have investigated if and how chemical reactions can be influenced by targeted vibrational excitation of the reactants. They were able to demonstrate that excitation with a laser beam does not affect the efficiency of a chemical exchange reaction and that the excited molecular group acts only as a spectator in the reaction.

A frequently used reaction in organic chemistry is nucleophilic substitution. It plays, for example, an important role in in the synthesis of new chemical...

Im Focus: New 2D Spectroscopy Methods

Optical spectroscopy allows investigating the energy structure and dynamic properties of complex quantum systems. Researchers from the University of Würzburg present two new approaches of coherent two-dimensional spectroscopy.

"Put an excitation into the system and observe how it evolves." According to physicist Professor Tobias Brixner, this is the credo of optical spectroscopy....

All Focus news of the innovation-report >>>

Anzeige

Anzeige

VideoLinks
Industry & Economy
Event News

Leading experts in Diabetes, Metabolism and Biomedical Engineering discuss Precision Medicine

13.07.2018 | Event News

Conference on Laser Polishing – LaP: Fine Tuning for Surfaces

12.07.2018 | Event News

11th European Wood-based Panel Symposium 2018: Meeting point for the wood-based materials industry

03.07.2018 | Event News

 
Latest News

A smart safe rechargeable zinc ion battery based on sol-gel transition electrolytes

20.07.2018 | Power and Electrical Engineering

Reversing cause and effect is no trouble for quantum computers

20.07.2018 | Information Technology

Princeton-UPenn research team finds physics treasure hidden in a wallpaper pattern

20.07.2018 | Materials Sciences

VideoLinks
Science & Research
Overview of more VideoLinks >>>