Study suggests new treatments for Huntington's disease

January 9, 2008

Working with fruit flies, researchers have discovered a new mechanism by which the abnormal protein in Huntington’s disease causes neurodegeneration. They have also manipulated the flies to successfully suppress that neurodegeneration, which they said suggests potential treatments to delay the onset and progression of the disease in humans.

Juan Botas and colleagues published their findings in the January 10, 2008, issue of the journal Neuron, published by Cell Press.

Huntington’s disease is caused by a mutation in the gene for the huntingtin protein (htt) that causes a genetic “stutter”—an abnormally long number of repeats of the amino acid glutamine at one end of the protein.

Previous studies had concentrated on the toxicity that the abnormal protein produces by forming cell-clogging aggregates in the nuclei of neurons. Most studies in animals, however, had not involved introducing the gene for full-length htt; they involved only a fragment.

In their experiments, Botas and colleagues introduced the gene for full-length abnormal human htt into the fruit fly Drosophila and studied its early effects on neural function in the flies.

They found that, before the abnormal protein produced any toxic effects in the nuclei of neurons, it caused abnormally high transmission of chemical signals, called neurotransmitters, among neurons. Such neurotransmitters are launched by one neuron across connections, called synapses, to its neighbor, triggering a nerve impulse in the receiving neuron. Besides abnormal synaptic transmission, the researchers also found that mutant htt caused neurodegeneration and degeneration in the flies’ motor ability.

The researchers found that they could suppress these abnormalities by introducing other mutations into the fly genome that either reduced neurotransmission or reduced the activity of pores called calcium channels in the membranes of neurons. Such channels trigger neurotransmission by controlling the influx of calcium into neurons.

“The findings described in this report unveil a mechanism of pathogenesis for expanded htt that does not require its nuclear accumulation in detectable amounts,” concluded the researchers. They wrote that the increased neurotransmission they detected “likely represents a mechanism of pathogenesis taking place at early stages of disease progression.

“These findings point to increased synaptic transmission as a therapeutic target with the potential of delaying [Huntington’s disease] onset and thus likely impacting disease progression,” they wrote. They concluded that their ability to genetically suppress the abnormal neurotransmission and neurodegeneration “further define[s] specific therapeutic targets and support[s] the idea that Ca2+ channel antagonists, and perhaps other inhibitors of neurotransmission, offer an attractive therapeutic option due to their specificity and wide usage.”

Source: Cell Press


Rank not rated yet
Tags

Relevant PhysicsForums posts
  • We the immaterial soul
    created5 hours ago
  • Is Everyday Technology Killing Us?
    createdFeb 08, 2012
  • Exercise and weight loss
    createdFeb 08, 2012
  • Why do we have head aches? Our brains can't feel anything.
    createdFeb 07, 2012
  • "The end of diseases" by David Agus, interview from Daily Show with Jon Stewart
    createdFeb 04, 2012
  • Oncolytic adenovirus
    createdFeb 04, 2012
  • More from Physics Forums - Medical Sciences

More news stories

Study finds that anti-diabetic medication can prevent the long-term effects of maternal obesity

In a study to be presented today at the Society for Maternal-Fetal Medicine's annual meeting, The Pregnancy Meeting, in Dallas, Texas, researchers will report findings that show that short therapy with the anti-diabetic medication ...

Medicine & Health / Health

created 23 hours ago | popularity 5 / 5 (1) | comments 1

FDA-approved drug rapidly clears amyloid from the brain, reverses Alzheimer's symptoms in mice

Neuroscientists at Case Western Reserve University School of Medicine have made a dramatic breakthrough in their efforts to find a cure for Alzheimer's disease. The researchers' findings, published in the journal Science, show t ...

Medicine & Health / Neuroscience

created Feb 09, 2012 | popularity 4.9 / 5 (53) | comments 21 | with audio podcast

Green tea found to reduce disability in the elderly

(Medical Xpress) -- A lot of research has been done over the past several years looking into the health benefits of green tea. As a result, scientists have found that regular consumption of the beverage leads ...

Medicine & Health / Health

created Feb 07, 2012 | popularity 4.4 / 5 (14) | comments 11 | with audio podcast report

Teen school drop-outs three times as likely to be on benefits in later life

Teen school drop-outs are almost three times as likely to be on benefits in later life as their peers who complete their schooling, indicates research published online in the Journal of Epidemiology and Community Health.

Medicine & Health / Health

created Feb 06, 2012 | popularity not rated yet | comments 12

To perform with less effort, practice beyond perfection

Whether you are an athlete, a musician or a stroke patient learning to walk again, practice can make perfect, but more practice may make you more efficient, according to a surprising new University of Colorado Boulder study.

Medicine & Health / Neuroscience

created Feb 09, 2012 | popularity 4.4 / 5 (15) | comments 6 | with audio podcast


Google might launch Drive for cloud storage soon

(PhysOrg.com) -- Google's next big move, according to the Wall Street Journal, is a cloud storage service called Drive. Hardly first to the plate, Google is simply catching up to introducing its cloud reposi ...

Latin America mining boom clashes with conservation

Latin America is experiencing a mining boom as prices rise fuelled by a hike in global demand, but the region is also being hit by a wave of violent protests, strikes and rallies by environmentalists.

Walney offshore wind farm is world's biggest (for now)

(PhysOrg.com) -- The Walney wind farm on the Irish Sea--characterized by high tides, waves and windy weather--officially opened this week. The farm is treated in the press as a very big deal as the Walney ...

Love a click away in Indonesia's Twitter Republic

He was a geeky kid from Yogyakarta, she a glamorous city girl in Jakarta. In a country with one of the world's most vibrant social networking scenes they fell in love on Twitter.

GPS court ruling leaves US phone tracking unclear

A US Supreme Court decision requiring a warrant to place a GPS device on the car of a criminal suspect leaves unresolved the bigger issue of police tracking using mobile phones, legal experts say.

Europeans protest controversial Internet pact

Tens of thousands of people marched in protests in more than a dozen European cities Saturday against a controversial anti-online piracy pact that critics say could curtail Internet freedom.