Forum for Science, Industry and Business

Sponsored by:     3M 
Search our Site:

 

Hebrew U.-Johns Hopkins study of nuclear envelope defects provides clues to dealing with human disease

31.08.2005


A step towards understanding cell mutations that cause a variety of human diseases, particularly in children -including that which brings about premature aging and early death - has been taken by researchers at the Hebrew University of Jerusalem Silberman Institute of Life Sciences and the John Hopkins University School of Medicine.



The scientists have focused their research on a study of induced mutations in the nuclear envelope of cells from the tiny C. elegans worm. Their aim is to thus provide clues towards a better understanding of mutations in proteins of the envelope of the cell nucleus in humans.

Such mutations, particularly in lamin (nuclear envelope) proteins A and C, cause many different diseases, including Hutchison Gilford progeria syndrome. Children with this disease develop premature aging and die usually before the age of 13. Other diseases brought about by these mutations include a form of muscular dystrophy, cardiomyopathy (a weakening of the heart muscle), and various other forms of irregular or retarded growth in childhood.


A report on the lamin research project was published in a recent issue of the Proceedings of the National Academy of Sciences in the U.S. The project was carried out primarily by Ayelet Margalit, a doctoral student in genetics at the Hebrew University, working under the supervision of Prof. Yosef Gruenbaum, and in cooperation with Prof. Katherine L. Wilson and Dr. Miriam Segura-Totten of Johns Hopkins University.

Experimenting with removal of the worm’s lamin protein or its interacting protein partners emerin, MAN1 or BAF, the researchers have described “down-the-line” consequences, including the disruption of various proteins necessary for normal cell reproduction. Even though the C. elegans worm has only one lamin protein and few proteins that interact with it, the processes that occur there are similar to what happens in humans and provide clues to the laminopathic diseases affecting people..

The results seen from these lamin complex disruptions are a halted process of cell division, resulting in a static “bridge” structure between cells that should have separated, plus damage to the gonad cell structure. In both cases, the ability of the organism to grow and to reproduce is severely impaired.

The researchers hope that through further laboratory experimentation with the worm they will be able to better understand the functions of lamin-based complexes, and why mutations in these proteins cause a variety of different laminopathic diseases, such as progeria and muscular dystrophy in humans.

Jerry Barach | alfa
Further information:
http://www.huji.ac.il

More articles from Life Sciences:

nachricht Molecular Force Sensors
20.09.2017 | Max-Planck-Institut für Biochemie

nachricht Foster tadpoles trigger parental instinct in poison frogs
20.09.2017 | Veterinärmedizinische Universität Wien

All articles from Life Sciences >>>

The most recent press releases about innovation >>>

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

Im Focus: Tiny lasers from a gallery of whispers

New technique promises tunable laser devices

Whispering gallery mode (WGM) resonators are used to make tiny micro-lasers, sensors, switches, routers and other devices. These tiny structures rely on a...

Im Focus: Ultrafast snapshots of relaxing electrons in solids

Using ultrafast flashes of laser and x-ray radiation, scientists at the Max Planck Institute of Quantum Optics (Garching, Germany) took snapshots of the briefest electron motion inside a solid material to date. The electron motion lasted only 750 billionths of the billionth of a second before it fainted, setting a new record of human capability to capture ultrafast processes inside solids!

When x-rays shine onto solid materials or large molecules, an electron is pushed away from its original place near the nucleus of the atom, leaving a hole...

Im Focus: Quantum Sensors Decipher Magnetic Ordering in a New Semiconducting Material

For the first time, physicists have successfully imaged spiral magnetic ordering in a multiferroic material. These materials are considered highly promising candidates for future data storage media. The researchers were able to prove their findings using unique quantum sensors that were developed at Basel University and that can analyze electromagnetic fields on the nanometer scale. The results – obtained by scientists from the University of Basel’s Department of Physics, the Swiss Nanoscience Institute, the University of Montpellier and several laboratories from University Paris-Saclay – were recently published in the journal Nature.

Multiferroics are materials that simultaneously react to electric and magnetic fields. These two properties are rarely found together, and their combined...

Im Focus: Fast, convenient & standardized: New lab innovation for automated tissue engineering & drug

MBM ScienceBridge GmbH successfully negotiated a license agreement between University Medical Center Göttingen (UMG) and the biotech company Tissue Systems Holding GmbH about commercial use of a multi-well tissue plate for automated and reliable tissue engineering & drug testing.

MBM ScienceBridge GmbH successfully negotiated a license agreement between University Medical Center Göttingen (UMG) and the biotech company Tissue Systems...

Im Focus: Silencing bacteria

HZI researchers pave the way for new agents that render hospital pathogens mute

Pathogenic bacteria are becoming resistant to common antibiotics to an ever increasing degree. One of the most difficult germs is Pseudomonas aeruginosa, a...

All Focus news of the innovation-report >>>

Anzeige

Anzeige

Event News

“Lasers in Composites Symposium” in Aachen – from Science to Application

19.09.2017 | Event News

I-ESA 2018 – Call for Papers

12.09.2017 | Event News

EMBO at Basel Life, a new conference on current and emerging life science research

06.09.2017 | Event News

 
Latest News

Molecular Force Sensors

20.09.2017 | Life Sciences

Producing electricity during flight

20.09.2017 | Power and Electrical Engineering

Tiny lasers from a gallery of whispers

20.09.2017 | Physics and Astronomy

VideoLinks
B2B-VideoLinks
More VideoLinks >>>