A new genetic model for a motor disorder that confines an estimated 10,000 people in the United States to walkers and wheelchairs indicates that instability in the microscopic scaffolding within a key set of nerve cells is the cause of this devastating disability. The study, which is published in the July 13 issue of the journal Current Biology, provides a provocative new insight into the molecular basis of the disease called hereditary spastic paraplegia (HSP) and suggests a new way to treat the inherited genetic disorder.
HSP--also known as familial spastic paraparesis and Strumpell-Lorrain syndrome--causes the ends of the nerves that control muscle activity to deteriorate. These nerve cells run from the brains cerebral cortex to the spinal cord where they connect to "downstream" nerve cells that excite muscles throughout the body to control coordinated movement. HSP causes weakness, spasms and loss of function in the muscles in the lower extremities.
More than 20 genes have been linked to HSP. However, more than 40 percent of all cases have been traced to a single gene (SPG4) that produces an enzyme called spastin. Previous studies have shown that this enzyme interacts with microtubules, the tiny protein tubes that provide structural support and transport avenues within most cells. Microtubules are dynamic structures, continually growing and shrinking, and their stability is closely regulated by a number of associated proteins. In nerve cells, microtubules carry cellular components to distant regions of the cell, regulate the growth of cellular branches and provide a substrate for important protein interactions. All of these functions are critically dependent on dynamic changes in microtubule stability.
David F. Salisbury | EurekAlert!
Many cooks don't spoil the broth: Manifold symbionts prepare their host for any eventuality
14.10.2019 | Max-Planck-Institut für Marine Mikrobiologie
Diagnostics for everyone
14.10.2019 | Max-Planck-Institut für Kolloid- und Grenzflächenforschung
A new research project at the TH Mittelhessen focusses on the development of a novel light weight design concept for leisure boats and yachts. Professor Stephan Marzi from the THM Institute of Mechanics and Materials collaborates with Krake Catamarane, which is a shipyard located in Apolda, Thuringia.
The project is set up in an international cooperation with Professor Anders Biel from Karlstad University in Sweden and the Swedish company Lamera from...
Superconductivity has fascinated scientists for many years since it offers the potential to revolutionize current technologies. Materials only become superconductors - meaning that electrons can travel in them with no resistance - at very low temperatures. These days, this unique zero resistance superconductivity is commonly found in a number of technologies, such as magnetic resonance imaging (MRI).
Future technologies, however, will harness the total synchrony of electronic behavior in superconductors - a property called the phase. There is currently a...
How do some neutron stars become the strongest magnets in the Universe? A German-British team of astrophysicists has found a possible answer to the question of how these so-called magnetars form. Researchers from Heidelberg, Garching, and Oxford used large computer simulations to demonstrate how the merger of two stars creates strong magnetic fields. If such stars explode in supernovae, magnetars could result.
How Do the Strongest Magnets in the Universe Form?
A hot, molten Earth would be around 5% larger than its solid counterpart. This is the result of a study led by researchers at the University of Bern. The difference between molten and solid rocky planets is important for the search of Earth-like worlds beyond our Solar System and the understanding of Earth itself.
Rocky exoplanets that are around Earth-size are comparatively small, which makes them incredibly difficult to detect and characterise using telescopes. What...
Scientists at the Max Planck Institute for Chemical Physics of Solids in Dresden, Princeton University, the University of Illinois at Urbana-Champaign, and the University of the Chinese Academy of Sciences have spotted a famously elusive particle: The axion – first predicted 42 years ago as an elementary particle in extensions of the standard model of particle physics.
The team found signatures of axion particles composed of Weyl-type electrons (Weyl fermions) in the correlated Weyl semimetal (TaSe₄)₂I. At room temperature,...
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