Huntington's Disease

New primate model for Huntington’s will help researchers

John Carroll — Tue, 20 May 2008 09:28:46 -0400

In a first, researchers have developed a primate model for Huntington's disease, eliciting harsh criticism from animal rights experts even as it paves the way for future primate models for a host of degenerative brain disorders. Five rhesus macaque monkeys now produce the rogue protein that leads to Huntington's. And the researchers say the work should help significantly advance work on a cure.

Mice have been used in neurodegenerative research, but don't demonstrate the same kind of symptoms that scientists look for. With the engineered monkeys, researchers will be able to get a much better understanding of the disease and better ways it can be treated in humans.

- read the story from New Scientist

Wellcome Trust plans spike in research spending

Tue, 05 Feb 2008 06:59:59 -0500

The UK's Wellcome Trust says it will pump up its research spending by 60 percent over the next five years as it ambitiously seeks a better understanding of diseases at a genetic level. The funding spike--to £4 billion-backs a range of research projects, including the Biobank project, which is seeking a greater understanding of genetics and environmental factors as well as the Case Control Consortium, which hunts for genes involved in disease.

"One of our priorities is to increase our understanding of common human diseases at the genetic level," Wellcome chief Dr Mark Walport told the BBC. "Developments in technology have meant we can now do this kind of science on a scale that we've not been able to before."

- see this release
- read the article from the BBC

ALSO: President Bush's new budget is keeping the NIH's funding at an even level, a fact that quickly aroused protests from groups that have been harshly critical of the long-term effects of flat-line research budgets. Report

Related Articles:
Research group mapping 1,000 genomes. Report
Cells prompted to "eat" Huntington proteins. Report

Cells prompted to "eat" Huntington proteins

Mon, 07 May 2007 20:01:39 -0400

Scientists have developed a novel strategy for tackling neurodegenerative diseases such as Huntington's disease: encouraging an individual's own cells to "eat" the malformed proteins that lead to the disease. Professor David Rubinsztein, Wellcome Trust Senior Clinical Fellow at the University of Cambridge, has been studying the molecular biology underlying Huntington's and other neurodegenerative diseases. Huntington's occurs when a protein known as huntingtin builds up in the brain cells of patients, mainly in neurons in the basal ganglia and in the cerebral cortex. Normally, cells dispose of or recycle their waste material, including unwanted or mis-folded proteins, through a process known as autophagy, or "self-eating".

"We have shown that stimulating autophagy in the cells--in other words, encouraging the cells to eat the malformed huntingtin proteins -- can be an effective way of preventing them from building up," says Professor Rubinsztein. "This appears to stall the onset of Huntington's-like symptoms in fruit fly and mice, and we hope it will do the same in humans."

- read the report on the research study

Related Articles:
Mayo researchers find trigger for Huntington's. Report
Huntington's study reveals insights on genetic testing. Report
New agent disables genetic diseases. Report

New five-gene test for lung cancer proves effective

Wed, 03 Jan 2007 19:01:39 -0500

Scientists at Taiwan University have developed a five-gene test that can help illuminate which lung cancer patients are likely to benefit from chemotherapy. Standard therapy calls for chemotherapy after surgery to remove a tumor, but some early-stage patients may not gain much benefit from chemo. Others have such aggressive cases that they're unlikely to benefit either.

The scientific team led by Hsuan-Yu Chen studied 125 patients and identified 16 genes that figured into their risk profile. They then narrowed that down to five genes that could be used to gain a clear picture of a person's risks. Specialists in the field say it's time to use the same process employed in breast cancer to identify which patients should be provided chemotherapy.

- check out the AP report for more information

ALSO: Researchers at Duke have developed a method to load fat particles with chemotherapy drugs, delivering targeted quantities of the therapy. Report

Related Articles:
Genetic testing advances as insurers balk. Report
Huntington's study reveals insights on genetic testing. Report

Researchers study role of glia in neurodegeneration

Wed, 30 Aug 2006 20:01:39 -0400

Researchers at the University of Washington say they've found that the tissue that surrounds neurons in the central nervous system--known as glia-plays a role in neurodegeneration. Using a mouse model, the scientists found that the reduced transfer of glutamate into glial cells played a role in the degeneration of Purkinje neurons. The theory is that that may be the cause of neuronal death seen in diseases like Alzheimer's, Huntington's and prion disease. Their focus was on spinocerebellar ataxia type 7, or SCA7, in which a mutation glia interferes with the transport of a neurotransmitter. Their work is published in the October issue of Nature Neuroscience.

- check out the UPI report on glia

Huntington's study reveals insights on genetic testing

Wed, 09 Aug 2006 20:01:39 -0400

Working with a team of scientists, neurologist Ira Shoulson, M.D., of the University of Rochester Medical Center has been undertaking a study of Huntington's disease that provides some real insight into how people will respond to a wave of genetic tests that are being developed and marketed. The group is repeatedly testing a group of 1,001 people who are at high risk of developing Huntington's, an incurable disease. These people, who have at least one parent who had the disease, have a 50 percent chance of developing it themselves. Interestingly, though, while a blood test exists to see if they have the gene that causes the disease, only one in ten of people who are at risk chose to be tested. The purpose of the test is to find therapies to alleviate or postpone the development of the disease. The team doesn't know why, but more than twice as many women as men have signed up to take part in the study.

- here's the report from EurekAlert for more information

PLUS: While we're on the subject of genetic testing, researchers say that they have developed one that reliably predicts whether the most common form of lung cancer will occur after surgery. Article

ALSO NOTED: Rapid cell death with apoptosis protein release; Electrical currents alter cell movement;

Wed, 02 Aug 2006 20:01:38 -0400

More Research News

> Researchers at St. Jude Children's Research Hospital in Memphis, TN say that they have found that cell death occurs as a rapid event that occurs with the almost simultaneous release of apoptosis proteins. Researchers had believed that cell death was a sequential event. Apoptosis, they say, swiftly eliminates extraneous, damaged or harmful cells. Article

> A scientific team says that they have identified two genes that trigger cell response to electricity and demonstrated that electrical currents can alter cell movement and speed healing. Article

> Researchers say that different genes may trigger autism in boys and girls, and that different genes may also be responsible for early-onset and late-onset autism. Article

> Researchers at the MassGeneral Institute for Neurodegenerative Disease have identified an enzyme that works to repair damaged cells as a potential target for Huntington's disease. Inhibiting Poly (ADP-ribose) polymerase (PARP1)--which works to repair cells but can in turn kill them when over-activated--may prove a method for treating Huntington's. Article

> CV Therapeutics says that the Journal of Clinical Investigation has published a preclinical study suggesting that CVT-6883 significantly reduced elevated markers of inflammation, fibrosis and pulmonary injury in two separate in vivo models. Release

> Researchers have been struggling to discover why testicular cancer is easier to treat than other metastatic cancers. One working theory by a group of researchers at Johns Hopkins is exploring the "hyperthermic stress response," in which germ cells that make sperm are retained at lower temperatures in the scrotum and die when exposed to normal body temperature. If they're right, they could identify new ways to treat other cancers. Article

> A scientific team at Glasgow's Beatson Institute for Cancer Research has found that mouth cancer develops in two distinct ways. In the most aggressive form of the disease, faults were found in the p53 gene with no expression of the p16 gene that regulates cells. In the first way, cells kept dividing and multiplying, spreading the disease. In the second way, cells were less likely to divide and multiply, making the disease less likely to spread. Article

> A new mouse model has been developed that provides clues why a gene therapy used to treat the rare "Bubble Boy" disease sometimes causes the patient to develop leukemia. Article

> Researchers at the University of Pittsburgh School of Medicine have concluded that gout is linked to a higher risk of heart attacks. Article

> Citizens groups have been waging a battle against a $178 million bioweapons lab in south Boston, saying they could be exposed to the lethal pathogens that would be studied there. Article

Tools and Technology

> Keith Elliston, the CEO of Genstruct, talks with Bio-IT World about the upsurge in systems biology and how it is likely to influence drug development work. Article

> PharmaWeek looks at the problems and potential of ongoing stem cell research programs and the different strategies drug developers have taken to position themselves in this field. Article

> Government agencies are raising alarms about direct-to-consumer DNA tests. Article

> SuperArray Bioscience has released the RT2Profiler PC Arrays, which now covers 36 different human and mouse pathways, or disease states. Article

> Bruker Daltonics has inked a distribution deal for Isis Pharmaceuticals' Ibis T5000, a universal biosensor system that can simultaneously identify thousands of types of infectious organisms in a sample. Release

Deals and Dollars

> The Foundation for Fighting Blindness will fund preclinical studies of RetinoStat for vision loss at Oxford Biomedica. Article

> Javelin Pharmaceuticals has been selected to receive a $750,000 grant from the National Cancer Institute/U.S. National Institutes of Health to advance clinical development of PMI-150 (intranasal ketamine) for pain control. Article

> Australia's Hunter Medical Research Institute has received a $6.6 million from the government to support its research work in cancer, heart disease and mental illness. Article

And Finally… Scientists say bumblebees use the color of plants to learn how to find the warm nectar they like the most. Article