A chemical found in plants could reduce the symptoms of a rare muscle disease that leaves children with little or no control of their movements.
Scientists have found that a plant pigment called quercetin – found in some fruits, vegetables, herbs and grains – could help to prevent the damage to nerves associated with the childhood form of motor neuron disease.
Their findings could pave the way for new treatments for spinal muscular atrophy (SMA) – also known as floppy baby syndrome – which is a leading genetic cause of death in children.
The team has found that the build-up of a specific molecule inside cells – called beta-catenin – is responsible for some of the symptoms associated with the condition.
In tests on zebrafish, flies and mice, scientists found that treating the disease with purified quercetin – which targets beta-catenin – led to a significant improvement in the health of nerve and muscle cells.
Quercetin did not prevent all of the symptoms associated with the disorder but researchers hope that it could offer a useful treatment option in the early stages of disease.
They now hope to create better versions of the chemical that are more effective than naturally-occurring quercetin.
SMA is caused by a mutation in a gene that is vital for the survival of nerve cells that connect the brain and spinal cord to the muscles, known as motor neurons. Until now, it was not known how the mutation damages these cells and causes disease.
The study reveals that the mutated gene affects a key housekeeping process that is required for removing unwanted molecules from cells in the body. When this process doesn't work properly, molecules can build-up and cause problems inside the cells.
Children with SMA experience progressive muscle wastage and loss of mobility and control of their movements. The disorder is often referred to as 'floppy baby syndrome' because of the weakness that it creates.
It affects one in 6000 babies and around half of children with the most severe form will die before the age of two.
The study is published today in the Journal of Clinical Investigation.
Professor Tom Gillingwater from the University of Edinburgh, who led the study, said: "This is an important step that could one day improve quality of life for the babies affected by this condition and their families. There is currently no cure for this kind of neuromuscular disorder so new treatments that can tackle the progression of disease are urgently needed."
Jen Middleton | EurekAlert!
A new potential biomarker for cancer imaging
05.02.2016 | Universiti Putra Malaysia (UPM)
NIH researchers identify striking genomic signature shared by 5 types of cancer
05.02.2016 | NIH/National Human Genome Research Institute
Automobiles increase the mobility of their users. However, their maneuverability is pushed to the limit by cramped inner city conditions. Those who need to...
Advance in biomedical imaging: The University of Würzburg's Biocenter has enhanced fluorescence microscopy to label and visualise up to nine different cell structures simultaneously.
Fluorescence microscopy allows researchers to visualise biomolecules in cells. They label the molecules using fluorescent probes, excite them with light and...
NASA's follow-on to the successful ICESat mission will employ a never-before-flown technique for determining the topography of ice sheets and the thickness of sea ice, but that won't be the only first for this mission.
Slated for launch in 2018, NASA's Ice, Cloud and land Elevation Satellite-2 (ICESat-2) also will carry a 3-D printed part made of polyetherketoneketone (PEKK),...
In the last decades, sea level has been rising continuously – about 3.3 mm per year. For reef islands such as the Maldives or the Marshall Islands a sinister picture is being painted evoking the demise of the island states and their cultures. Are the effects of sea-level rise already noticeable on reef islands? Scientists from the ZMT have now answered this question for the Takuu Atoll, a group of Pacific islands, located northeast of Papua New Guinea.
In the last decades, sea level has been rising continuously – about 3.3 mm per year. For reef islands such as the Maldives or the Marshall Islands a sinister...
The ‘Internet of Things’ is growing rapidly. Mobile phones, washing machines and the milk bottle in the fridge: the idea is that minicomputers connected to these will be able to process information, receive and send data. This requires electrical power. Transistors that are capable of switching information with a single electron use far less power than field effect transistors that are commonly used in computers. However, these innovative electronic switches do not yet work at room temperature. Scientists working on the new EU research project ‘Ions4Set’ intend to change this. The program will be launched on February 1. It is coordinated by the Helmholtz-Zentrum Dresden-Rossendorf (HZDR).
“Billions of tiny computers will in future communicate with each other via the Internet or locally. Yet power consumption currently remains a great obstacle”,...
02.02.2016 | Event News
26.01.2016 | Event News
26.01.2016 | Event News
05.02.2016 | Life Sciences
05.02.2016 | Materials Sciences
05.02.2016 | Physics and Astronomy