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 Cnidarians remotely control bacteria
21.09.2017 | Christian-Albrechts-Universität zu Kiel

nachricht Immune cells may heal bleeding brain after strokes
21.09.2017 | NIH/National Institute of Neurological Disorders and Stroke

All articles from Life Sciences >>>

The most recent press releases about innovation >>>

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

Im Focus: Highly precise wiring in the Cerebral Cortex

Our brains house extremely complex neuronal circuits, whose detailed structures are still largely unknown. This is especially true for the so-called cerebral cortex of mammals, where among other things vision, thoughts or spatial orientation are being computed. Here the rules by which nerve cells are connected to each other are only partly understood. A team of scientists around Moritz Helmstaedter at the Frankfiurt Max Planck Institute for Brain Research and Helene Schmidt (Humboldt University in Berlin) have now discovered a surprisingly precise nerve cell connectivity pattern in the part of the cerebral cortex that is responsible for orienting the individual animal or human in space.

The researchers report online in Nature (Schmidt et al., 2017. Axonal synapse sorting in medial entorhinal cortex, DOI: 10.1038/nature24005) that synapses in...

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...

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

Comet or asteroid? Hubble discovers that a unique object is a binary

21.09.2017 | Physics and Astronomy

Cnidarians remotely control bacteria

21.09.2017 | Life Sciences

Monitoring the heart's mitochondria to predict cardiac arrest?

21.09.2017 | Health and Medicine

VideoLinks
B2B-VideoLinks
More VideoLinks >>>