LONDON: Scientists at the University College, London, have developed a way of reversing the early symptoms of prion diseases like the human form of mad cow disease, variant Creutzfeldt-Jakob disease, or vCJD, and the disease in cattle called bovine spongiform encephalopathy.
The findings suggest that a way can be found to treat the disease in humans if it is detected before it had done harm to a large number of brain cells.
The scientists, led by Giovanna Malucci at the Medical Research Council of the college, have been able to reverse the early symptoms of mad cow disease in mice. The researchers infected the mice with abnormal prions and found they began to develop the disease, but when the mice stopped producing their own prions, the symptoms cleared up. This goes on to suggest that blocking the body's ability to make its own natural, healthy prions reversed early symptoms of the disease.
Prions or rogue proteins, affect the brain cells, changing their shape, creating holes and turning them "spongy". Malucci and her team have reported in the journal Neuron how they halted this chain reaction by genetically switching off production of normal prion proteins. The mice had been genetically altered to generate an enzyme in their bodies around nine weeks of age, which removed the gene that makes prion. Mice which continued to produce normal prions became increasingly ill and died.
Malucci explains that if there is no natural prion protein present in the brain, the conversion process to the rogue form cannot take place and damage to brain cells will be prevented.
Malucci feels the results could be helpful in developing a treatment for vCJD or other prion diseases. However, as the results show, the success will depend on how early the disease was detected.
Scientists will also need to know more about the exact role of prions in the system, she said adding the MRC unit has already taken a first step by identifying drugs that bind to normal prions.
