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.
"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.
Erik Sandelin; Anna Nordlund; Peter M. Andersen; Stefan S. L. Marklund; Mikael Oliveberg, Stockholm University.
Maria Erlandsson | alfa
Nonstop Tranport of Cargo in Nanomachines
20.11.2018 | Max-Planck-Institut für molekulare Zellbiologie und Genetik
Researchers find social cultures in chimpanzees
20.11.2018 | Universität Leipzig
Max Planck researchers revel the nano-structure of molecular trains and the reason for smooth transport in cellular antennas.
Moving around, sensing the extracellular environment, and signaling to other cells are important for a cell to function properly. Responsible for those tasks...
Researchers at the University of New Hampshire have captured a difficult-to-view singular event involving "magnetic reconnection"--the process by which sparse particles and energy around Earth collide producing a quick but mighty explosion--in the Earth's magnetotail, the magnetic environment that trails behind the planet.
Magnetic reconnection has remained a bit of a mystery to scientists. They know it exists and have documented the effects that the energy explosions can...
Biochips have been developed at TU Wien (Vienna), on which tissue can be produced and examined. This allows supplying the tissue with different substances in a very controlled way.
Cultivating human cells in the Petri dish is not a big challenge today. Producing artificial tissue, however, permeated by fine blood vessels, is a much more...
Faster and secure data communication: This is the goal of a new joint project involving physicists from the University of Würzburg. The German Federal Ministry of Education and Research funds the project with 14.8 million euro.
In our digital world data security and secure communication are becoming more and more important. Quantum communication is a promising approach to achieve...
On Saturday, 10 November 2018, the research icebreaker Polarstern will leave its homeport of Bremerhaven, bound for Cape Town, South Africa.
19.11.2018 | Event News
09.11.2018 | Event News
06.11.2018 | Event News
20.11.2018 | Life Sciences
20.11.2018 | Life Sciences
20.11.2018 | Physics and Astronomy