Scientists have created a new mouse model for spinal and bulbar muscular atrophy (SBMA), a disease characterized by adult-onset progressive weakness and degeneration of limb muscles, often resulting in the patient being confined to a wheel chair. SBMA causes the death of cells called motor neurons that control muscle function. The study, published in the March 4 issue of Neuron, presents a clearer picture of the pathology underlying SBMA and associated diseases and even points to a possible therapeutic strategy for this debilitating condition and for more common motor neuron diseases, such as amyotrophic lateral sclerosis (ALS), that currently have no proven treatments.
SBMA belongs to a group of neurodegenerative disorders, called polyglutamine diseases, that includes Huntingtons disease and spinocerebellar ataxias. Polyglutamine diseases are thought to arise because of a mutant protein that is misfolded and subsequently clumps together to form toxic aggregates that destroy cell function and cause disease. In SBMA, a mutated gene directs production of androgen receptors with an abnormal number of consecutive residues of the amino acid glutamine. Dr. Albert R. La Spada and colleagues from the University of Washington Medical Center in Seattle created transgenic mice containing the human androgen receptor carrying 100 glutamine repeats. The mice developed a gradually progressive limb weakness around mid-adulthood that was accompanied by motor neuron degeneration, strikingly similar to what is seen in human SBMA patients. The researchers determined that the abnormal androgen receptor interfered with production of a molecule called vascular endothelial growth factor (VEGF) that is important for the general health and survival of motor neurons. Interestingly, VEGF could rescue SBMA-like motor neurons grown in the laboratory.
The researchers conclude that VEGF may play a pivotal role in motor neuron degeneration. "Our findings in SBMA suggest that activation of the VEGF pathway may be one way that the motor neuron protects itself from harmful insults and stresses. Studies of ALS (amyotrophic lateral sclerosis) also point to the VEGF axis as critical for motor neuron health, so it is distinctly possible that all motor neuron diseases share interruption of the VEGF axis as part of their pathogenesis," explains Dr. La Spada. "If this is true, then it would have dramatic implications for treatment of motor neuron diseases."
Heidi Hardman | EurekAlert!
Inactivate vaccines faster and more effectively using electron beams
23.03.2017 | Fraunhofer-Institut für Organische Elektronik, Elektronenstrahl- und Plasmatechnik FEP
Hunting pathogens at full force
22.03.2017 | Helmholtz-Zentrum für Infektionsforschung
Astronomers from Bonn and Tautenburg in Thuringia (Germany) used the 100-m radio telescope at Effelsberg to observe several galaxy clusters. At the edges of these large accumulations of dark matter, stellar systems (galaxies), hot gas, and charged particles, they found magnetic fields that are exceptionally ordered over distances of many million light years. This makes them the most extended magnetic fields in the universe known so far.
The results will be published on March 22 in the journal „Astronomy & Astrophysics“.
Galaxy clusters are the largest gravitationally bound structures in the universe. With a typical extent of about 10 million light years, i.e. 100 times the...
Researchers at the Goethe University Frankfurt, together with partners from the University of Tübingen in Germany and Queen Mary University as well as Francis Crick Institute from London (UK) have developed a novel technology to decipher the secret ubiquitin code.
Ubiquitin is a small protein that can be linked to other cellular proteins, thereby controlling and modulating their functions. The attachment occurs in many...
In the eternal search for next generation high-efficiency solar cells and LEDs, scientists at Los Alamos National Laboratory and their partners are creating...
Silicon nanosheets are thin, two-dimensional layers with exceptional optoelectronic properties very similar to those of graphene. Albeit, the nanosheets are less stable. Now researchers at the Technical University of Munich (TUM) have, for the first time ever, produced a composite material combining silicon nanosheets and a polymer that is both UV-resistant and easy to process. This brings the scientists a significant step closer to industrial applications like flexible displays and photosensors.
Silicon nanosheets are thin, two-dimensional layers with exceptional optoelectronic properties very similar to those of graphene. Albeit, the nanosheets are...
Enzymes behave differently in a test tube compared with the molecular scrum of a living cell. Chemists from the University of Basel have now been able to simulate these confined natural conditions in artificial vesicles for the first time. As reported in the academic journal Small, the results are offering better insight into the development of nanoreactors and artificial organelles.
Enzymes behave differently in a test tube compared with the molecular scrum of a living cell. Chemists from the University of Basel have now been able to...
20.03.2017 | Event News
14.03.2017 | Event News
07.03.2017 | Event News
22.03.2017 | Materials Sciences
22.03.2017 | Physics and Astronomy
22.03.2017 | Materials Sciences