Huntington’s disease research news. In plain language. Written by scientists. For the global HD community.
Clumps of mutant huntingtin protein in brain cells are a hallmark of HD, and they build up slowly, occupying more and more cells over time. Recent research in mice shows that the harmful proteins can travel between neurons, setting off a chain reaction that leads to more sick cells and the development of symptoms.
It’s like gardening gone wrong: scientists can sprinkle Huntington’s protein on the outside of laboratory-grown brain cells and make sticky, potentially harmful protein clumps grow inside the cells. Now, new research showing that human brain fluid does the same thing could help us monitor Huntington's disease.
New work in brain diseases like Alzheimer's suggests that brain cells called neurons might be 'catching' the sickness from their neighbors. A recently published paper suggests that, in very specific lab conditions, this might also happen in Huntington's disease. What does this mean for what we know about HD, and how to treat it?
We know that the cause of Huntington's disease is a genetic change, resulting a harmful protein: mutant huntingtin. But other proteins can get dragged into the fray and contribute to the problems faced by HD-affected cells. New research suggests that a rather notorious protein, called 'tau' – a known troublemaker in other degenerative brain diseases – builds up and causes damage in HD.
Huntington's disease is caused by the malfunctioning and early death of brain cells. Replacing those dead and dying cells with stem cells has long been a goal of some HD scientists. A new study investigates the long-term health of some of the earliest cell transplants into HD patient brains — and finds a surprising result.
A chemical called cysteamine has long been of interest as a potential therapy for Huntington's disease. Now, Raptor Pharmaceuticals has announced the interim results of a study of cysteamine in HD patients. The trial failed to meet its pre-specified goal, but there are some interesting details in the data suggesting the ongoing trial deserves attention.
Studies have shown that HD patients tend to get less efficient sleep, fewer hours of sleep, and wake up more times during the night. However, sleep in Huntington’s is under-researched because historically scientists have investigated HD as a disease of movement impairment, and sleep problems don’t seem to have anything to do with movement impairment.
Copper, the metal, may play a role in worsening the symptoms of Huntington’s disease. Bing Zhou and his team looked for connections between HD and the amount of copper in neurons. They report that reducing copper in neurons or keeping it from binding to the HD protein improves symptoms.
DNA is the longest instruction manual on Earth. Because it's so long, cells use special helper proteins called HDACs to shut down sections of the manual they don’t use very often. Now, scientists have shown that interfering with one specific HDAC improves HD-related problems in cells and mice — but does so in an unexpected way.