(Press-News.org) Scientists from The University of Manchester say a naturally occurring chemical found in soy could prove to be an effective new treatment for a fatal genetic disease that affects children.
Dr Brian Bigger, from the University's MPS Stem Cell Research Laboratory, found that genistein – derived from soya beans and licensed in the US as an osteoporosis drug – had a dramatic effect on mice suffering from the human childhood disease Sanfilippo.
"Sanfilippo is an untreatable mucopolysaccharide (MPS) disease affecting one in 89,000 children in the United Kingdom," said Dr Bigger, who is based in the School of Biomedicine.
"Children with Sanfilippo disease experience progressive deterioration of mental function, similar to dementia, in early childhood, with other symptoms including severe behavioural problems, hyperactivity and ultimately death in early teens."
In the study, published in the journal Public Library of Science One, mice with Sanfilippo disease were fed with high doses of genistein over a nine-month period. Treated mice showed a significant delay in their mental decline, including a third reduction in the amount of excess sugars found in the brain as a result of the disease, and a sixth reduction in inflammation in the brain.
Importantly, the research, carried out with colleagues at St Mary's Hospital in Manchester, also showed that the hyperactivity and other abnormal behaviour normally seen in Sanfilippo mice were fully corrected by genistein treatment.
Professor Wraith, a co-author on the study and consultant paediatrician from Genetic Medicine in St Mary's Hospital, said "Sanfilippo is a disease where the genetic lack of an enzyme leads to a fault in the breakdown of complex sugars in the cell.
"This leads to storage of these undegraded complex sugars in cells, disturbances in brain function and ultimately to this profound mental deterioration that we see in the children with this condition. Manchester is a specialist centre for this type of genetic disease and as such we look after more than 100 patients from all over the UK and beyond."
The Manchester team, supported by the UK society for mucopolysaccharide diseases and the Manchester Biomedical Research Centre, hope to announce a placebo controlled clinical trial for patients with Sanfilippo disease in the near future.
INFORMATION:
Notes for editors:
A copy of the paper is available on request.
More information can be found at:
http://www.medicine.manchester.ac.uk/geneticmedicine/research/genetics/biochemical/
http://www.mpssociety.co.uk/ (Family support contact: Christine Lavery)
http://www.medicine.manchester.ac.uk/staff/BrianBigger
Soya beans could hold clue to treating fatal childhood disease
2010-12-04
ELSE PRESS RELEASES FROM THIS DATE:
Smoking may thin the brain
2010-12-04
Philadelphia, PA, 2 December 2010 - Many brain imaging studies have reported that tobacco smoking is associated with large-scale and wide-spread structural brain abnormalities.
The cerebral cortex is a specific area of the brain responsible for many important higher-order functions, including language, information processing, and memory. Reduced cortical thickness has been associated with normal aging, reduced intelligence, and impaired cognition.
However, prior research had not described the impact of smoking upon cortical thickness.
A new study, published in ...
Low-status leaders are ignored
2010-12-04
People who are deemed social misfits or "losers" aren't effective leaders, even if they are crusading for a cause that would benefit a larger group, according to new research from Rice University, the University of Texas and Universitat de Valencia.
The study's authors observed the contributions of 80 participants in a repeated public-goods game and found that players were more likely to mimic the actions of a leader they perceived as a high-status individual; they ignored leaders perceived as low-status and, when they had a chance, punished them for trying to lead. ...
Do our bodies' bacteria play matchmaker?
2010-12-04
Tel Aviv ― Could the bacteria that we carry in our bodies decide who we marry? According to a new study from Tel Aviv University, the answer lies in the gut of a small fruit fly.
Prof. Eugene Rosenberg, Prof. Daniel Segel and doctoral student Gil Sharon of Tel Aviv University's Department of Molecular Microbiology and Biotechnology recently demonstrated that the symbiotic bacteria inside a fruit fly greatly influence its choice of mates.
The research was done in cooperation with Prof. John Ringo of the University of Maine, and was recently published in the Proceedings ...
Widely used arthritis pill protects against skin cancer
2010-12-04
A widely-used arthritis drug reduces the incidence of non-melanoma skin cancers – the most common cancers in humans – according to a study published this week in the Journal of the National Cancer Institute. The COX-2 inhibitor celecoxib (brand name Celebrex), which is currently approved for the treatment of osteoarthritis, rheumatoid arthritis and acute pain in adults led to a 62 percent reduction in non-melanoma skin cancers, which includes basal cell carcinomas and squamous cell carcinomas.
Celecoxib, a prescription-strength nonsteroidal anti-inflammatory drug (NSAID), ...
Kicking the habit: Study suggests that quitting smoking improves mood
2010-12-04
PROVIDENCE, R.I. [Brown University] — Quitting smoking is certainly healthy for the body, but doctors and scientists haven't been sure whether quitting makes people happier, especially since conventional wisdom says many smokers use cigarettes to ease anxiety and depression. In a new study, researchers tracked the symptoms of depression in people who were trying to quit and found that they were never happier than when they were being successful, for however long that was.
Based on their results, the authors of the article published online Nov. 24 in the journal Nicotine ...
Set of specific interventions rapidly improves hospital safety 'culture'
2010-12-04
A prescribed set of hospital-wide patient-safety programs can lead to rapid improvements in the "culture of safety" even in a large, complex, academic medical center, according to a new study by safety experts at Johns Hopkins.
"It doesn't take decades or tons of money to get from a culture that says 'mistakes are inevitable' to a belief that harm is entirely preventable," says Peter Pronovost, M.D., Ph.D., a professor of anesthesiology and critical care medicine at the Johns Hopkins University School of Medicine and leader of the study published online in the journal ...
Relationship-strengthening class improves life for new families
2010-12-04
Expectant parents who completed a brief relationship-strengthening class around the time their child was born showed lasting effects on each family member's well being and on the family's overall relationships, according to a recent Penn State study.
The team, led by Mark Feinberg, senior research associate in Penn State's Prevention Research Center for the Promotion of Human Development, analyzed the effects of the Family Foundations program for three years after a child was born.
The Family Foundations program, offered in several Pennsylvania locations as part of ...
Lower occurrence of atopic dermatitis in children thanks to farm animals and cats
2010-12-04
Atopic dermatitis (also known as atopic eczema) is a chronic and extremely painful inflammation of the skin that frequently occurs in early childhood, generally already in infancy. Up to 20 percent of all children in industrialized countries are affected, making it one of the most common childhood skin diseases.
The need to better understand this disease is all the greater considering the intense suffering it causes in small children. Atopic dermatitis is, however, an allergic condition and all allergic reactions result from complex interactions of genetic and environmental ...
Checklist continues to stop bloodstream infections in their tracks, this time in Rhode Island
2010-12-04
Using a widely heralded Johns Hopkins checklist and other patient-safety tools, intensive care units across the state of Michigan reduced the rate of potentially lethal bloodstream infections to near zero.
Now, led by the same Johns Hopkins patient-safety expert who spearheaded the Michigan program, researchers in Rhode Island have shown the Michigan results weren't just a fluke.
The new study, published in the December issue of the journal Quality and Safety in Health Care, found that the rate of central-line associated bloodstream infections (CLABSIs) fell by 74 ...
Beyond nature vs. nurture: Parental guidance boosts child's strengths, shapes development
2010-12-04
Why does a child grow up to become a lawyer, a politician, a professional athlete, an environmentalist or a churchgoer?
It's determined by our inherited genes, say some researchers. Still others say the driving force is our upbringing and the nurturing we get from our parents.
But a new child-development theory bridges those two models, says psychologist George W. Holden at Southern Methodist University in Dallas. Holden's theory holds that the way a child turns out can be determined in large part by the day-to-day decisions made by the parents who guide that child's ...