Navigation Links
Gladstone scientists identify protein form linked to Huntington's disease

SAN FRANCISCO, CAOctober 30, 2011Scientists at the Gladstone Institutes have discovered how a form of the protein linked to Huntington's disease influences the timing and severity of its symptoms, offering new avenues for treating not only this disease, but also a variety of similar conditions.

In a paper being published today in Nature Chemical Biology, the laboratory of Gladstone Senior Investigator Steven Finkbeiner, MD, PhD, singles out one form of a misfolded protein in neurons that best predicts whether the neuron will die. Neuronal death is key to the development of Huntington's symptomsincluding erratic behavior, memory loss and involuntary muscle movement. This research underscores the value of the cross-disciplinary work done at Gladstonea leading and independent biomedicalresearch organizationwhile revealing techniques that scientists anywhere can apply to conditions involving misfolded proteins, such as Alzheimer's disease and type 1 diabetes.

"Effective treatments for diseases such as Huntington's and Alzheimer's have been slow to develop," said Dr. Finkbeiner, whose research at Gladstone investigates the interactions between genes, neurons and memory. "We hope that our newfound understanding of precisely which misfolded proteins contribute to disease symptoms will speed up drug development for sufferers."

Huntington's, an ultimately fatal disease that affects more than a quarter of a million people nationwide, is caused by mutations in the gene that creates the huntingtin, or htt, protein. As the mutated gene produces htt, a segment of the protein called polyglutamine is mistakenly expanded, distorting htt's natural shape and function. As a result, the misfolded protein malfunctions and can be toxic.

Previous research into Huntington's found that misfolded proteins in the brain lead to the dysfunction and subsequent death of neurons. In this study, Dr. Finkbeiner set out to find which misfolded htt types were the most toxic. In laboratory experiments on mice, he and his colleagues screened numerous antibodies that each bind uniquely to one type of misfolded htt in order to identify and tag each type. These antibodies acted as molecular tracking devices that Dr. Finkbeiner monitored with an automated microscope and specialized software, both of which his lab created. Experiments revealed that one of the antibodies, 3B5H10, bound to a form of htt closely linked to neuron death and, therefore, disease progression.

"Now that our experiments have identifiedat a cellular levelwhen neurons will die, it will be easier to develop drugs that target the toxic form of htt that causes Huntington's symptoms," said Dr. Finkbeiner, who is also a professor of neurology and physiology at the University of California, San Francisco (UCSF), with which Gladstone is affiliated.

"Dr. Finkbeiner's powerful and precise approach could enable the development of pharmaceutical treatments for other diseases as well," added Lennart Mucke, MD, who directs neurological research at Gladstone. "These methods are applicable to many devastating neurodegenerative conditions, especially Parkinson's disease and Alzheimer's."


Contact: Anne Holden
Gladstone Institutes

Related biology news :

1. Gladstone scientist finds new target for treating symptoms of Parkinsons disease
2. Gladstone scientists offer new insight into the regulation of stem cells and cancer cells
3. Gladstone to receive $5.6 million in federal funds to seek a cure for AIDS
4. Gladstone scientists identify genes involved in embryonic heart development
5. Gladstone scientists uncover mechanism for the major genetic risk factor of Alzheimers disease
6. Gladstone scientists link hepatitis C virus infection to fat enzyme in liver cells
7. Gladstones Shinya Yamanaka wins Kyoto prize
8. Gladstone and Institute for Systems Biology collaborate on Huntingtons disease
9. Gladstones Shinya Yamanaka wins Lasker Award
10. Gladstone scientists identify key factors in heart cell creation
11. Gladstone scientists reveal key enzyme in fat absorption
Post Your Comments:
Related Image:
Gladstone scientists identify protein form linked to Huntington's disease
(Date:4/15/2016)... 2016  A new partnership announced today will ... decisions in a fraction of the time it ... high-value life insurance policies to consumers without requiring ... Force Diagnostics, rapid testing (A1C, Cotinine and HIV) ... pressure, weight, pulse, BMI, and activity data) available ...
(Date:4/14/2016)... 14, 2016 BioCatch ™, ... today announced the appointment of Eyal Goldwerger ... Goldwerger,s leadership appointment comes at a time ... the deployment of its platform at several of the ... which discerns unique cognitive and physiological factors, is a ...
(Date:3/31/2016)... March 31, 2016   ... or the "Company") LegacyXChange is excited to ... its soon to be launched online site for trading ... ) will also provide potential shareholders a sense ... technology to an industry that is notorious for fraud. ...
Breaking Biology News(10 mins):
(Date:6/23/2016)... ... June 23, 2016 , ... STACS DNA Inc., the sample ... the Arkansas State Crime Laboratory, has joined STACS DNA as a Field Application Specialist. ... said Jocelyn Tremblay, President and COO of STACS DNA. “In further expanding our capacity ...
(Date:6/23/2016)... NEWPORT BEACH, Calif. , June 23, 2016 /PRNewswire/ ... offering new biological discoveries to the medical community, has ... and co-founder Matthew Nunez . "We ... provide us with the capital we need to meet ... funding will essentially provide us the runway to complete ...
(Date:6/23/2016)... ... June 23, 2016 , ... ClinCapture, the only free validated ... will showcase its product’s latest features from June 26 to June 30, 2016 ... on Disrupting Clinical Trials in The Cloud during the conference. DIA (Drug ...
(Date:6/23/2016)... June 22, 2016  Amgen (NASDAQ: AMGN ... QB3@953 life sciences incubator to accelerate the ... shared laboratory space at QB3@953 was created to help ... obstacle for many early stage organizations - access to ... sponsorship, Amgen launched two "Amgen Golden Ticket" awards, providing ...
Breaking Biology Technology: