Fatal childhood disease treated with soya

Soya beans could hold the key to treating a fatal childhood disease say researchers in Manchester, who have been testing a derivative of the bean on mice.

Sanfilippo disease causes a dementia-like mental decline, behavioural problems and an early death in affected children, but scientists believe some of the symptoms can be slowed or corrected with a derivative of the soya bean – genistein.

“Sanfilippo is an untreatable mucopolysaccharide (MPS) disease affecting one in 89,000 children in the United Kingdom,” said Dr Brian Bigger from the University of Manchester’s MPS Stem cell research Laboratory.

Bigger and colleagues from St Mary’s Hospital in Manchester fed mice with Sanfilippo disease high doses of genistein – a naturally occuring isoflavone and antioxidant – over a period of nine months.

“Sanfilippo is a disease where the genetic lack of an enzyme leads to a fault in the breakdown of complex sugars in the cell,” said Professor Wraith, a consultant paediatrician from St Mary’s Hospital, “This leads to storage of these ungraded complex sugars in cells, disturbances in brain function and ultimately to this profound mental deterioration that we see in the children with this condition.”

Researchers found the 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. The research also showed that hyperactivity and other abnormal behaviour normally seen in Sanfilippo mice were fully corrected by genistein treatment.

The Manchester team – supported by the UK Society for Mucopolysaccharide Diseases and the Manchester Biomedical Research Medical Centre – hope to announce a placebo controlled clinical train for patients with the disease in the near future.