Instagram Twitter Facebook HD Forum Donate and Get Involved

Sheep model of Huntington’s Disease reveals brain pathology before the onset of symptoms

Scientists from New Zealand and Australia have been using sheep to study Huntington’s Disease (HD). Until at least six years of age, these HD sheep don’t lose any brain cells. Even at ten years of age, HD sheep have no movement problems. However, they do have early signs of HD. These include problems such as a build-up of Huntington’s protein in their brain, changes to their day-night activity cycle and changes to many small molecules found in the brain, liver and blood.

Dysfunction in the Middle Cingulate Cortex is associated with the mood symptoms of HD

Huntington’s Disease (HD) can cause a range of movement, mood and cognitive symptoms. Some people have more of some symptoms than others, and we still don’t know exactly why. We know that damage to a part of the brain called the Anterior Cingulate Cortex (or ACC for short) is associated with the mood symptoms of HD. The cingulate cortex is part of the cortex, which is the layer that wraps around the outside of the brain and looks like a walnut. The cingulate cortex is near the middle of the cortex, close to where the two halves of the cortex meet. It has roles in regulating mood and some aspects of thinking. The Middle Cingulate Cortex (MCC) is right next to the ACC. Because it also plays a role in mood, it is possible that damage to this part of the brain is also responsible for creating some of the mood symptoms of HD.

New Zealand Scientists share data from an HD sheep model with the global scientific community

Despite scientists discovering the gene for Huntington’s Disease (HD) in 1993, it’s still unclear how this gene causes HD. Animals that have been genetically modified to have Huntington’s Disease are the best hope of understanding this disease and finding a cure. An international team of scientists, led by New Zealand’s very own, Professor Russell Snell from the University of Auckland, have been using these sheep to help solve the mystery of HD.

Fasting and Ketogenic Diets in Huntington’s Disease

Brain and muscle cells require a ton of energy compared with most other cells, and nearly all that energy is generated by hundreds of “batteries” within each cell, called mitochondria. However, these mitochondria are quite damaged in Huntington’s Disease, which might be caused by mutant huntingtin protein. Thus, one way to treat Huntington’s may be to use a “genetic” strategy to create normal versions of the huntingtin protein, which might improve the mitochondria, leading to more energy for the brain and muscle cells. However, there could be another way we could treat Huntington’s…by using a “metabolic” strategy to improve the mitochondria directly.

Demystifying Enroll-HD

You might have heard of this thing called Enroll-HD, but don’t really know what it’s all about, what it means to participate, or who you will be seeing at the study clinic. We bring you this blog post written by Auckland-based study coordinator Christina Buchanan on why she loves being part of Enroll-HD and what to expect from your visits to the study centre.

Investigating the Link between Smell and Huntington’s disease

In many people an early indicator of Huntington’s disease are changes to their smell or olfaction. The process of olfaction works through structures in our nose known as the nasal pathway, and in our brain known as the olfactory system. The main proponents to this system are the olfactory bulbs, which are neural structures that receive smell information and send it to other regions of the brain to be processed.

Untangling the Secrets of Huntington's Disease Epigenetics

Our genes are made up of DNA. DNA can be thought of as an ‘instruction manual’ to make proteins to build us. However, in Huntington’s disease the DNA ‘instruction manual’ differs and the proteins built may allow for Huntington’s symptoms to present, such as involuntary movement and rigidity. What people may not be as aware of is that epigenetics can also play a role in disease. Epigenetics refers to the factors which can alter the expression of genes. To put this simply, if DNA is the’ instruction manual’, epigenetics can help determine what can be read within the manual.

The Cerebellum in Huntington's disease

The cerebellum is a region of the brain involved in coordination, fine-motor movement, posture, and balance, all of which have been shown to be affected in Huntington’s disease. Neurological research of Huntington’s disease has predominantly focused on the basal ganglia and cortical areas in the brain, due to the damage and loss of cell in these regions. The role of the cerebellum in Huntington’s disease is currently unclear, as various studies have produced different findings. A group of New Zealand researchers at the Centre for Brain Research set out to explain the role of the cerebellum in Huntington’s disease, and whether it varies between the motor and mood sub-groups.

Kiwis unlocking secrets from deep inside the Huntington's brain

Researchers know that when the medium spiny neurons start to die, the globus pallidus as a whole begins to shrink, but they hadn’t figured out why it was shrinking. In this research, scientists were able to examine each part of the globus pallidus and determine which parts were being affected and how they were being affected.