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Oz Buzz Updates: Day 3

Our final daily report from the Huntington’s disease World Congress brings together all the live updates from our twitter feed. Video of both Oz Buzz sessions – with news, interviews and features – is on YouTube now and will be available to watch at HDBuzz.net later this week.

Wednesday, September 14, 2011

8:33 – Jeff and Ed are now live from day 3 of the World Congress on HD!

8:40 – Jeff: HD may be much more common than we thought – Michael Hayden

8:47 – Jeff: HD is an increasing burden on the elderly, who in the past may not have lived long enough to have symptoms – Hayden

9:02 – Ed: More on increased ‘prevalence’ of HD – how it’s more common than we thought

9:07 – Ed: studies in sperm helping to predict whether a CAG repeat in the ‘gray area’ will cause problems for next generation

9:13 – We need to move away from talking about ‘onset’ in Huntington’s – symptoms begin gradually over years – Dr Mark Guttman

9:32 – Huntington’s disease causes a wide variety of symptoms so doctors should always bear it in mind when seeing patients- Elizabeth McKusker

10:36 – Ed’s reporting from the ‘Science: omics’ session – Jeff’s in ‘International models of care’.

10:37 – Ed: ‘omics’ means measuring loads of things at once. Like genomics (looking at tons of genes)

10:45 – Ed: Metabolomics is measuring metabolites – small molecules in blood. Wayne Matson’s done it in HD & finds interesting changes

10:54 – Ed: Levels of a chemical I3PA seem to be reduced in blood of HD patients and HD mice. Not yet clear why but could be useful – Matson

11:02 – Jeff: HD patient care in Australia is difficult because of complex health care administration – Andrew Churchyard

11:00 – Jeff: Significant numbers of HD families don’t interact with the medical system, and we don’t know why – Churchyard

11:11 – Ed: Brain immune cells called microglia are abnormal in HD mice – Dr Blair Leavitt – are they helping or harming?

11:13 – Ed: There are also changes in the mice’s brain blood vessels that could increase the cross-talk between brain & body – Leavitt

11:26 – Jeff: Access to medical services for HD families in South Africa is limited – Amanda Krause

11:34 – Jeff: In black Africans a disease called ‘HD like 2’ looks very much like HD, but is caused by a different mutation – Krause

11:38 – Ed: Ruth Luthi-Carter examines which genes are more and less activated in different mouse models of HD & compares them to humans

11:51 – Ed: These gene activation changes could help us to understand the disease and develop and test new drugs – Luthi-Carter

12:06 – Ed: Proteins do stuff by sticking to each other. Mutant and normal huntingtin stick to different groups of partners – Chris Ross

12:11 – Ed: An international consortium of researchers is using stem cell models to help understand Huntington’s disease

12:18 – Jeff: Francisco Cardoso – new Latin american HD network active at rlah.org

12:20 – Ed: The consortium has figured out the recipe for turning stem cells into the neurons most affected by HD. Very valuable research tool

12:27 – Red Latino-Americana de Huntington: investigación de tratamientos efectivos para la Enfermedad de Huntington

12:43 – Ed: Comparing gene switching and behavior across mouse models shows that different models mimic different aspects of HD Lesley Jones

12:44 – Ed: One of the mouse models called Q150 actually produces less huntingtin protein overall – weird but important to know about – Jones

12:45 – Ed: An HDBuzz article on the different mice used in Huntington’s disease research is coming soon

13:47 – Ed and Jeff are now reporting from the final science session – late breaking hot topics. Jeff is the first speaker in the session!

13:56 – Jeff studies metabolites in several tissues in HD mice. Tissues like brain, fat and liver are all changed differently by the mutation.

14:00 – Metabolic changes Jeff found in HD mouse blood mirror the changes in the brain – could be useful for studying human patients

14:19 – Dimebon showed no benefit for HD in big trial- HORIZON. The quest for treatments for thinking probs continues.

14:21 – However, HORIZON study recruited rapidly and was run efficiently – good news for future trials of upcoming treatments – Bernhard Landwehrmeyer

14:42 – Chemical ‘tattoos’ are added to DNA by enzymes. HD messes this up. HDAC inhibitor drugs should help – a trial’s underway – Larry Marsh

14:50 – There are many different DNA ‘tattoos’ = many ways to try to improve things with drugs. Animal studies will identify best ones – Marsh

15:10 – Jeff: Clare van Eyk uses fruit flies to try and understand how the mutated huntingtin gene kills brain cells

15:12 – ‘RNA’, as well as protein might contribute to brain cells dying – Clare

15:13 – Jennifer Thompson is studying the psychiatric symptoms of HD, like apathy and depression, which can be devastating

15:16 – Apathy is incredibly common in HD, and worsens over time – Thompson

15:18 – Interestingly, depression is also common in HD, but doesn’t seem to change much over time – Thompson

15:36 – Robi Blumenstein of CHDI – like a chess game, we need to think far ahead if we’re going to beat Huntington’s disease

15:40 – Success is a three-legged stool: (1) an effective treatment (2) the ability to test it and (3) enough trial volunteers – Blumenstein

16:00 – Lots of HD family members needed for trials, now & future. Find out how to get involved at Enroll-HD.org

Latest Research Articles

Regulating repetition: Gaining control of CAG repeats could slow progression of Huntington’s disease

Published date: 30 November, 2023

“Somatic expansion” is a hot topic in Huntington’s disease research. Somatic expansion is a process in which CAG repeats lengthen in some cells during aging. It’s thought to control how early HD symptoms appear. A group of researchers from Toronto, Canada recently identified proteins that may play an important role in regulating this process. Understanding ... Read more

Getting to the Root of Huntington's Disease: A Plant-Based Approach

Published date: 15 October, 2023

Researchers studied a fragment of the Huntington’s disease (HD) protein in plants and found a new way to stop it from forming toxic clumps. A special plant protein that the team identified can prevent harmful buildup in plants as well as in some HD model systems, showing potential for this approach as a possible way ... Read more

Could halting CAG expansions be a new treatment for HD?

Published date: 5 October, 2023

A recent paper from a group at UMass Chan Medical School, spearheaded by Dr. Daniel O'Reilly and led by Dr. Anastasia Khvorova, used genetic strategies to lower a protein other than huntingtin. This time the researchers went after a gene called MSH3. This is a gene that’s been getting a lot of attention in Huntington’s ... Read more

Tipping the balance; new insights into HD genetic modifiers

Published date: 1 September, 2023

Genetic modifiers can influence when HD symptoms begin. Some of these genes encode for different types of molecular machines whose normal job is to repair our DNA when it is broken or damaged. A recently published study from scientists at Thomas Jefferson University uncovers details of how these molecular machines help repair damaged DNA structures ... Read more

Drug to treat movement symptoms of HD approved by FDA

Published date: 22 August, 2023

The vast majority of people with Huntington’s disease experience movement symptoms known as chorea. Valbenazine, also known as INGREZZA, has recently been approved by the United States Food and Drug Administration (FDA), allowing doctors in the USA to prescribe this medicine for Huntington’s disease (HD) chorea. In this article we go through the key points ... Read more

Youthful competitors: young brain cells oust the old

Published date: 8 August, 2023

When you lose something, an easy solution can be to just replace it. But what if the something you’ve lost are cells in the brain? Can they simply be replaced? Some researchers have been working toward this for Huntington’s disease (HD) by injecting new cells into the brains of animal models. A recent publication that ... Read more