An aggregating protein causes many of the symptoms of Huntington's disease, an incurable and frequently fatal brain disorder. Mosaic winner Ahmad Aziz discovered that the abnormal protein also aggregates in the hypothalamus, the part of the brain that regulates the autonomic nervous system and the endocrine system. Ahmad Aziz investigated the severity and causes of a number of less well-known symptoms of Huntington's disease: weight loss, sleep disturbances and a poorly functioning autonomic nervous system. He established that many patients suffer from weight loss and sleeping problems. Abnormalities in the hypothalamus appear to account for some of these symptoms. The hypothalamus was an obvious suspect for Aziz, as it regulates the autonomic nervous system and the endocrine system. Aziz established that certain nerve cells are lost in a part of the hypothalamus.
Huntington's disease (HD) is much more common in the UK than current estimates suggest, according to a new Comment published in the peer-reviewed medical journal The Lancet. The Comment was written by Professor Sir Michael Rawlins, Chairman of the UK National Institute for Health and Clinical Excellence (NICE). In this article Sir Michael writes in his capacity as an Honorary Professor of the London School of Hygiene and Tropical Medicine, UK. Huntington's disease is an incurable, hereditary brain disorder. It is a devastating brain disorder for which there is no currently 'effective' treatment. Nerve cells become damaged, causing various parts of the brain to deteriorate. The disease affects movement, behavior and cognition - the affected individuals' abilities to walk, think, reason and talk are gradually eroded to such a point that they eventually become entirely reliant on other people for their care.
Prana Biotechnology Limited (NASDAQ: PRAN) (ASX: PBT) announced that its Head of Research, Assoc. Prof. Robert Cherny, will present new data on PBT2, the Company's lead compound in development for Alzheimer's disease, at the Hot Topics Therapeutics/Intervention session on July 14th at the International Conference of Alzheimer's Disease (ICAD) in Honolulu. The presentation is entitled "Novel molecular mechanisms for the neurotrophic and neuroprotective effects of PBT2 in Alzheimer's disease and Huntington's disease ." The new findings show that the effectiveness of PBT2 lies in a unique combination of complementary activities. PBT2 acts to detoxify A-beta by disarming it of copper and zinc and returns these crucial metals to neurons. Assoc. Prof. Cherny will present data* showing that by returning these metals to neurons, important cell signaling pathways are activated that prevent neuronal death and promote neuronal function.
Rui Costa, Principal Investigator of the Champalimaud Neuroscience Programme at the Instituto Gulbenkian de CiĆ ncia (Portugal), and Xin Jin, of the National Institute on Alcohol Abuse and Alcoholism, National Institutes of Health (USA), describe in the latest issue of the journal Nature, that the activity of certain neurons in the brain can signal the initiation and termination of behavioural sequences we learn anew. Furthermore, they found that this brain activity is essential for learning and executing novel action sequences, many times compromised in patients suffering from disorders such as Parkinson's or Huntington's. Animal behaviour, including our own, is very complex and is many times seen as a sequence of particular actions or movements, each with a precise start and stop step.
The prevalence of Huntington's disease (HD) is substantially underestimated in the UK, with significant implications for those affected, the healthcare system, and research. New estimates of prevalence, and their implications, are discussed in a comment published in an upcoming Lancet, written by Professor Sir Michael Rawlins, who is the Chairman of the UK National Institute for Health and Clinical Excellence (NICE), but writes in his capacity as an Honorary Professor of the London School of Hygiene and Tropical Medicine, UK. HD is a progressive neurodegenerative disorder, which affects muscle coordination and leads to cognitive decline and dementia. Symptoms typically become noticeable in the fourth decade of life. HD is the most common genetic cause of involuntary writhing movements (called chorea), and is more common in people of Western European decent than in those from Asia or Africa.