14 Dec 2012, BioSpectrum Bureau , BioSpectrum
Singapore: Prana Biotechnology announced that the Journal of Huntington's Disease, has in its December 2012 edition, published data showing the benefits of the company's PBT2 in treating the disease.
The paper describes PBT2's ability to inhibit the development of the symptoms and pathological features of Huntington Disease in pre-clinical transgenic animal models. PBT2 is being trialed in Huntington Disease patients in 20 sites across the US and Australia. Results are expected in the second half of 2013.
"PBT2 markedly reduced neurodegeneration, significantly increased lifespan and improved motor function and co-ordination in an aggressive animal model of the disease," said lead author and head of research at Prana, Associate Professor Robert Cherny.
"It is already well established that PBT2 prevents the aggregation of the Abeta protein outside neurons, in Alzheimer's Disease. It is also established that the mutant Huntingtin (Htt) protein aggregates inside the neuron in Huntington Disease. There is published evidence that the protein aggregation in both diseases is driven by the interaction with metals. Our work has shown that PBT2 can prevent this protein aggregation caused by interaction with metals," he added.
At a recent New York Academy of Sciences symposium on targeting metals to treat neurodegenerative diseases, Professor Steven M Hersch, of Massachusetts General Hospital and Harvard Medical School commented that "Transition metals, especially iron and copper, have been implicated in the pathogenesis of Huntington Disease. Copper may directly modulate the toxicity of the Htt protein while iron accumulation in response to neurodegeneration likely potentiates the damage to the central nervous system, making both metals potential therapeutic targets. PBT2 is the first clinical candidate that modulates Htt directly."