Forum for Science, Industry and Business

Sponsored by:     3M 
Search our Site:

 

Hereditary ALS linked to low electric charge in cells

31.07.2007
Inside the body, our organs are elegantly kept apart by slick membranes. Inside our smallest components, our cells, a similar separation is upheld with the help of electrical charges. In the same way that reversed magnets repel each other, gauzes of negative charges prevent proteins, genetic material, and fats from sticking to each other in the wrong way.

In an article in the scientific journal The Journal of Biological Chemistry, Mikael Oliveberg, professor of biochemistry at Stockholm University in Sweden, describes how disturbances in these functions underlie the hereditary form of the motor-neuron disease Amyotrophic Lateral Sclerosis (ALS).

"Genetic studies have recently shown that even tiny disturbances in this balance of charges are one of the factors that cause the hereditary form of ALS. The disease is basically tied to the SOD1 protein suddenly starting to aggregate in small lumps in the nerve cells of the spinal cord and at the same time withering and dying. When this happens the musculature becomes paralyzed," says Mikeal Oliveberg.

Normally SOD1 proteins avoid this inappropriate lumping because their surfaces are adorned with some 40 negative charges. But if only one of these charges is lost, the disease is incurred-­the proteins can no longer remain soluble. A mystery in this context is that patients who were born with this faulty SOD1 protein remain fully healthy for their first 50-60 years of life. In some way the cells manage to compensate for the faulty proteins, but this capacity is eventually lost with aging.

... more about:
»Cells »Oliveberg »SOD1

"The goal is to be able to stimulate the built-in defense mechanisms that keep us healthy during the first half of our lives so that they have the vigor to keep working a few more years. To do this we need to learn more about why nerve death escalates so suddenly and, above all, so predictably at the molecular level," says Mikael Oliveberg.

Similar mechanisms underlie several other feared protein disorders like Alzheimer's and Parkinson's. The discovery that charges play such a critical role in ALS is an important step toward understanding these processes in a broader perspective.

"Another puzzle is why red deers seem to get along with an SOD1 protein that has a substantially lower negative charge than that in humans. Perhaps their cellular defense mechanisms are tuned differently, or could it be that old elks in fact have a higher propensity to perish from ALS-like symptoms? It would be interesting to hear whether anybody knows anything about this," says Mikael Oliveberg.

Amyotrophic Lateral Sclerosis-associated Copper/Zinc Superoxide Dismutase Mutations Preferentially Reduce the Repulsive Charge of the Proteins, The Journal of Biological Chemistry, Vol. 282, Issue 29, 21230-21236, JULY 20, 2007

Erik Sandelin; Anna Nordlund; Peter M. Andersen; Stefan S. L. Marklund; Mikael Oliveberg, Stockholm University.

Maria Erlandsson | alfa
Further information:
http://www.eks.su.se

Further reports about: Cells Oliveberg SOD1

More articles from Life Sciences:

nachricht Researchers uncover protein-based “cancer signature”
05.12.2016 | Universität Basel

nachricht The Nagoya Protocol Creates Disadvantages for Many Countries when Applied to Microorganisms
05.12.2016 | Leibniz-Institut DSMZ-Deutsche Sammlung von Mikroorganismen und Zellkulturen 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: Shape matters when light meets atom

Mapping the interaction of a single atom with a single photon may inform design of quantum devices

Have you ever wondered how you see the world? Vision is about photons of light, which are packets of energy, interacting with the atoms or molecules in what...

Im Focus: Novel silicon etching technique crafts 3-D gradient refractive index micro-optics

A multi-institutional research collaboration has created a novel approach for fabricating three-dimensional micro-optics through the shape-defined formation of porous silicon (PSi), with broad impacts in integrated optoelectronics, imaging, and photovoltaics.

Working with colleagues at Stanford and The Dow Chemical Company, researchers at the University of Illinois at Urbana-Champaign fabricated 3-D birefringent...

Im Focus: Quantum Particles Form Droplets

In experiments with magnetic atoms conducted at extremely low temperatures, scientists have demonstrated a unique phase of matter: The atoms form a new type of quantum liquid or quantum droplet state. These so called quantum droplets may preserve their form in absence of external confinement because of quantum effects. The joint team of experimental physicists from Innsbruck and theoretical physicists from Hannover report on their findings in the journal Physical Review X.

“Our Quantum droplets are in the gas phase but they still drop like a rock,” explains experimental physicist Francesca Ferlaino when talking about the...

Im Focus: MADMAX: Max Planck Institute for Physics takes up axion research

The Max Planck Institute for Physics (MPP) is opening up a new research field. A workshop from November 21 - 22, 2016 will mark the start of activities for an innovative axion experiment. Axions are still only purely hypothetical particles. Their detection could solve two fundamental problems in particle physics: What dark matter consists of and why it has not yet been possible to directly observe a CP violation for the strong interaction.

The “MADMAX” project is the MPP’s commitment to axion research. Axions are so far only a theoretical prediction and are difficult to detect: on the one hand,...

Im Focus: Molecules change shape when wet

Broadband rotational spectroscopy unravels structural reshaping of isolated molecules in the gas phase to accommodate water

In two recent publications in the Journal of Chemical Physics and in the Journal of Physical Chemistry Letters, researchers around Melanie Schnell from the Max...

All Focus news of the innovation-report >>>

Anzeige

Anzeige

Event News

ICTM Conference 2017: Production technology for turbomachine manufacturing of the future

16.11.2016 | Event News

Innovation Day Laser Technology – Laser Additive Manufacturing

01.11.2016 | Event News

#IC2S2: When Social Science meets Computer Science - GESIS will host the IC2S2 conference 2017

14.10.2016 | Event News

 
Latest News

High-precision magnetic field sensing

05.12.2016 | Power and Electrical Engineering

Construction of practical quantum computers radically simplified

05.12.2016 | Information Technology

NASA's AIM observes early noctilucent ice clouds over Antarctica

05.12.2016 | Earth Sciences

VideoLinks
B2B-VideoLinks
More VideoLinks >>>