New hope for Lou: Unexplored therapeutic targets for ALS
News Sep 04, 2015
The death of neurons from the neurodegenerative disorder amyotrophic lateral sclerosis (ALS) leads to muscle weakness that impacts breathing, movement and other physical functions. More commonly known as Lou Gehrig's disease, ALS has no cure, and the only approved therapy slows the progression by only a few months. In a new study presented at the 14th International Conference on Endothelin: Physiology, Pathophysiology and Therapeutics taking place September 2-5, 2015 in Savannah, Georgia, researchers from Johns Hopkins University have identified an unexplored avenue of treatment for ALS.
Endothelin (ET)-1, a small protein produced by blood vessel cells and a powerful vessel constrictor, is also produced by astrocytes, cells in the brain that studies are revealing play many roles in health and disease. ALS progression is associated with the dysfunction of astrocytes, and earlier studies have shown that ET-1 influences a number of cellular pathways implicated in ALS progression. Gene expression studies also suggest that levels of ET-1 and the receptor it binds to, ET-B, are elevated in patients with ALS.
This new study investigated whether levels of ET and its receptor, ET-B, were altered in the regions where nerve cells die in ALS. The researchers examined ALS patient-derived tissue samples and cells and a mouse model of ALS using a range of gene expression and protein measurement techniques. They found a higher level of ET-1 in astrocytes and a higher level of the ET-B receptor in nerve cells in regions affected in ALS. They also found variations in the gene sequences for ET-1 in patients with an inherited form of ALS.
"These experiments demonstrate striking abnormalities in the central nervous system endothelin system, [suggesting that] the endothelin system may represent a largely unexplored and potentially significant target for therapeutic intervention in ALS," according to the researchers.
Note: Material may have been edited for length and content. For further information, please contact the cited source.
A recent retrospective study evaluating continuous electroencephalography (cEEG) of children in intensive care units (ICUs) found a higher than anticipated number of seizures. The work also identified several conditions closely associated with the seizures, and suggests that cEEG monitoring may be a valuable tool for helping to identify and treat neurological problems in patients who are 14 months old or younger.
Pain is a negative feeling that we want to get rid of as soon as possible. In order to protect our bodies, we react for example by withdrawing the hand. This action is usually understood as the consequence of the perception of pain. A team from the Technical University of Munich (TUM) has now shown that perception, the impulse to act and provision of energy to do so take place in the brain simultaneously and not, as was expected, one after the other.