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!
A room with a view - or how cultural differences matter in room size perception
25.04.2017 | Max-Planck-Institut für biologische Kybernetik
Studying a catalyst for blood cancers
25.04.2017 | University of Miami Miller School of Medicine
More and more automobile companies are focusing on body parts made of carbon fiber reinforced plastics (CFRP). However, manufacturing and repair costs must be further reduced in order to make CFRP more economical in use. Together with the Volkswagen AG and five other partners in the project HolQueSt 3D, the Laser Zentrum Hannover e.V. (LZH) has developed laser processes for the automatic trimming, drilling and repair of three-dimensional components.
Automated manufacturing processes are the basis for ultimately establishing the series production of CFRP components. In the project HolQueSt 3D, the LZH has...
Reflecting the structure of composites found in nature and the ancient world, researchers at the University of Illinois at Urbana-Champaign have synthesized thin carbon nanotube (CNT) textiles that exhibit both high electrical conductivity and a level of toughness that is about fifty times higher than copper films, currently used in electronics.
"The structural robustness of thin metal films has significant importance for the reliable operation of smart skin and flexible electronics including...
The nearby, giant radio galaxy M87 hosts a supermassive black hole (BH) and is well-known for its bright jet dominating the spectrum over ten orders of magnitude in frequency. Due to its proximity, jet prominence, and the large black hole mass, M87 is the best laboratory for investigating the formation, acceleration, and collimation of relativistic jets. A research team led by Silke Britzen from the Max Planck Institute for Radio Astronomy in Bonn, Germany, has found strong indication for turbulent processes connecting the accretion disk and the jet of that galaxy providing insights into the longstanding problem of the origin of astrophysical jets.
Supermassive black holes form some of the most enigmatic phenomena in astrophysics. Their enormous energy output is supposed to be generated by the...
The probability to find a certain number of photons inside a laser pulse usually corresponds to a classical distribution of independent events, the so-called...
Microprocessors based on atomically thin materials hold the promise of the evolution of traditional processors as well as new applications in the field of flexible electronics. Now, a TU Wien research team led by Thomas Müller has made a breakthrough in this field as part of an ongoing research project.
Two-dimensional materials, or 2D materials for short, are extremely versatile, although – or often more precisely because – they are made up of just one or a...
20.04.2017 | Event News
18.04.2017 | Event News
03.04.2017 | Event News
25.04.2017 | Earth Sciences
25.04.2017 | Life Sciences
25.04.2017 | Earth Sciences