Cell Rep: new research reveals Huntington key protein

January 22, 2020 / Biovalley BIOON / -- a research team from Duke University found a new function of a gene called Huntington protein, whose mutation is the basis of the occurrence and deterioration of Huntington's disease Using a genetic mouse model, they found that neurons in the striatum that control movement need the Huntington gene to regulate the body's movement, maintain cell health and develop functional connections between cells during aging The researchers say studying the role of the gene in maintaining these neural connections may provide a new way to fight Huntington's disease Huntington's disease is a hereditary neurodegenerative disease that usually occurs in middle age and results in impaired motor control, dementia, and psychiatric symptoms Although the genetic mechanism of this fatal disease has been discovered more than 20 years ago, there is no approved treatment to slow its development or cure The disease is caused by a mutation in one of two copies of the Huntington gene The mutation results in an abnormal version of the Huntington protein, which is toxic to neurons Although the mutant protein is expressed in vivo, the neurons in striatum are particularly susceptible to it and degenerate with the development of the disease Although the mutant Huntington protein damages neurons, it may also interfere with the ability of the remaining non mutant Huntington protein to perform its normal function The drugs currently being tested in clinical trials are designed to block the defective Huntington protein, but will eventually reduce the content of normal Huntington protein in neurons It is well known that Huntington protein plays several important roles in cells, but its specific role in the health and function of striatal neurons is not clear In a study published in the journal Cell reports, the team found that specifically removing the Huntington gene from the striatal neurons of very young mice can cause these neurons to die as they age, similar to the pattern of neuron death in Huntington's chorea They also found that mice lacking Huntington's protein in striatal neurons were impaired in their ability to control movement Importantly, this dyskinesia occurs even before the neuron itself begins to die "These findings suggest that cell death itself may not be the only cause of symptoms of Huntington's disease," said Eroglu, author of the article In a healthy brain, striatal neurons communicate with other neurons through connections called synapses, which control movement The researchers found that striatal neurons lacking Huntington protein formed abnormal synaptic connections, which may explain the problem of motor function in mice "We believe that changes in neuron and synapse levels prior to cell death are contributing to disease development," Caley Burrus said Source of information: new roles found for Huntington's disease protein source: Caley J Burrus et al, strategic project neurons require huntingtin for synthetic connectivity and survival, cell reports (2020) Doi: 10.1016/j.cellep.2019.12.069