空泡tau蛋白病,痴呆症 z-bo 2020-10-16 501

　　In a new study, researchers at the University of Pennsylvania, the University of Athens, and the Perelman School of Medicine at the Athens Medical Center have discovered a new rare form of dementia. This discovery also reveals a new way of protein accumulation in the brain, which may be the target of developing new therapies. The accumulation of protein in the brain can lead to this newly discovered disease and related neurodegenerative diseases, such as Alzheimer's disease (AD). Alzheimer's disease is a neurodegenerative disease characterized by the accumulation of tau protein in specific parts of the brain. These researchers examined human brain tissue samples from donors who died of an unknown neurodegenerative disease and found new changes in the valoxin-containing protein (VCP) gene in the brain. Mutations, accumulation of tau protein in mutated brain regions and vacuoles that appear in neurons. They named the newly discovered disease Vacuolar Tauopathy (VT)-a neurodegenerative disease characterized by neural vacuoles and tau protein aggregates.

　　The corresponding author of this article, Dr. Edward Lee, assistant professor of pathology and experimental medicine at the Perelman School of Medicine at the University of Pennsylvania, said: "In cells, proteins are aggregated. They are blocked and do not work. The effect of VCP is Discover and isolate aggregated proteins. Mutations are the normal ability of these proteins to destroy protein aggregates."

　　These researchers pointed out that the accumulation of tau protein they observed is very similar to the accumulation of tau protein in Alzheimer's disease. With these similarities, their goal is to discover how this VCP mutation causes this new disease and help find a cure for this disease and other diseases. The genetic causes of rare diseases often provide new insights into more common diseases. In addition to studying cells and animal models, these researchers first studied the protein itself and found that the accumulation of tau protein in this newly discovered disease is actually caused by VCP mutations. "In this study, we found a pattern we have never seen before and a mutation that we have never explained. This mutation drives the activity of VCP. Given its inhibitory effect, this suggests that the opposite can also be done. Established-if you can increase the activity of VCP, it will help break down protein aggregates, which is correct. This very rare disease can not only break down tau protein aggregates, but also Alzheimer's disease and others Tau protein aggregates in diseases related to tau protein aggregates.