Navigation Links
Small changes in protein chemistry play large role in Huntington's disease
Date:12/24/2009

In Huntington's disease, a mutated protein in the body becomes toxic to brain cells. Recent studies have demonstrated that a small region adjacent to the mutated segment plays a major role in the toxicity. Two new studies supported by the National Institutes of Health show that very slight changes to this region can eliminate signs of Huntington's disease in mice.

Researchers do not fully understand why the protein (called mutant huntingtin) is toxic, but one clue is that it accumulates in ordered clumps of fibrils, perhaps clogging up the cells' internal machinery.

"These studies shed light on the structure and biochemistry of the mutant huntingtin protein and on potentially modifiable factors that affect its toxicity," said Margaret Sutherland, Ph.D., a program director at NIH's National Institute of Neurological Disorders and Stroke (NINDS). "They reveal sites within the huntingtin protein and within broader disease pathways that could serve as targets for drug therapy."

Both studies were published online this week. One study, published in the Journal of Cell Biology, was led by Leslie Thompson, Ph.D., and Joan Steffan, Ph.D., of the University of California, Irvine. The other study, in Neuron, was led by X. William Yang, M.D., Ph.D., of the University of California, Los Angeles in collaboration with Ron Wetzel, Ph.D., of the University of Pittsburgh School of Medicine.

Huntington's disease is inherited, and usually strikes in middle age, producing uncontrollable movements of the legs and arms, a loss of muscle coordination, and changes in personality and intellect. It is inexorably progressive and leads to death of affected persons usually within 20 years after symptoms first appear. Individuals with the disease carry mutations that affect the huntingtin protein. The mutations involve a triple repeat DNA sequence, a type of genetic miscue similarly found in Friedreich's ataxia, Kennedy's disease, fragile X s
'/>"/>

Contact: Gregory Roa
greg.roa@nih.gov
301-496-5751
NIH/National Institute of Neurological Disorders and Stroke
Source:Eurekalert

Page: 1 2 3

Related biology news :

1. Social parasites of the smaller kind
2. Small animal imaging facility is big boon to research
3. Handbook of small grain insects available now
4. New molecular clock from LLNL and CDC indicates smallpox evolved earlier than believed
5. 2007 ozone hole smaller than usual
6. Cilia: small organelles, big decisions
7. Research shows loggerhead sea turtles threatened by small-scale fishing operations
8. Small-scale fishing in Mexico rivals industrial fisheries in accidental turtle deaths
9. Sweet potato shines as new promise for small enterprise and hunger relief in developing countries
10. Identification of a novel class of (not-so) small RNAs
11. Small RNA plays parallel roles in bacterial metabolism
Post Your Comments:
*Name:
*Comment:
*Email:
(Date:12/15/2014)... , Dec. 12, 2014 Research and Markets ... the "Global Facial Recognition Market 2015-2019" report ... Facial recognition is a technology used ... the overall facial feature of a person such as ...
(Date:12/11/2014)... 2014  Data Sciences International (DSI), the global leader ... series of digital telemetry implants to meet the ... part of the PhysioTel™ Digital platform, was designed ... data when incorporating functional endpoints into repeat-dose toxicology ...
(Date:12/10/2014)... DIEGO , Dec. 9, 2014 CIE San Diego ... provides the connective tissue that enhances care coordination among social ... client-level information; earned a second $1 million grant from the ... in community and; will be recognized as a "Live Well ...
Breaking Biology News(10 mins):Global Facial Recognition Market 2015-2019: Key Vendors are 3M Cogent, Cognitec Systems, NEC and Safran Group 2Global Facial Recognition Market 2015-2019: Key Vendors are 3M Cogent, Cognitec Systems, NEC and Safran Group 3New telemetry implants expected to change how large animal toxicology studies are conducted 2Community Information Exchange San Diego Launches Pilot, Secures $1 Million Expansion Grant, and Becomes a "Live Well San Diego" Partner 2Community Information Exchange San Diego Launches Pilot, Secures $1 Million Expansion Grant, and Becomes a "Live Well San Diego" Partner 3Community Information Exchange San Diego Launches Pilot, Secures $1 Million Expansion Grant, and Becomes a "Live Well San Diego" Partner 4
... a group of human subjects to operate a robot-controlled joystick, ... "learns" to control certain muscle movements, the more likely it ... results, the investigators say, could alter rehabilitation approaches for people ... , In a report on the work in the ...
... and exercise performance measures of trained cyclists at high ... a lot -- up to 45% -- while others ... level. , Ten cyclists who took sildenafil at altitude ... kilometers by 15% compared to placebo trials at altitude. ...
... for breast cancer do not increase their life expectancy ... analysis by researchers from UC Davis, UCSF, the University ... addition, the researchers showed that tamoxifen is an extraordinarily ... per year of life saved. The study will be ...
Cached Biology News:New approach to vaccine development provides potent, long-lasting immunity 2New approach to vaccine development provides potent, long-lasting immunity 3Viagra improves high altitude exercise performance up to 45% for some 2Viagra improves high altitude exercise performance up to 45% for some 3Viagra improves high altitude exercise performance up to 45% for some 4Tamoxifen for breast cancer prevention does not benefit most women 2Tamoxifen for breast cancer prevention does not benefit most women 3
(Date:1/22/2015)... January 22, 2015 Pipette.com has added ... to their comprehensive portfolio of Eppendorf products. , The ... purchase a Centrifuge 5424/5424 R and receive the following:, ... Eppendorf Research plus or Eppendorf Reference 2 , ...
(Date:1/22/2015)... Selexis SA, a serial innovation company with proven ... for drug discovery to commercial manufacturing, announced today all ... Sequencing (NGS) data packages. The NGS analysis of ... the integrity of the gene, validation of the left ...
(Date:1/22/2015)...  Varian Medical Systems (NYSE: VAR ), world leader ... commitment to sustainability with inclusion on a prestigious list of ... healthcare equipment company among the Corporate Knights Global 100 Most ... at Davos, Switzerland . ...
(Date:12/24/2014)... Md. , Dec. 24, 2014  United Therapeutics Corporation ... Inc. (NYSE: MDT ) has submitted a ... Administration (FDA) for the use of Medtronic,s SynchroMed ® ... developed catheter) for use with United Therapeutics, Remodulin ® ...
Breaking Biology Technology:Eppendorf Announces Promotional Bundle Which Includes: Eppendorf Centrifuge 5424, 3-Pack of Pipettes, and Tips - Available Now at Pipette.com 2Selexis Generated Research Cell Banks Now Fully Sequenced Using Next-Generation Sequencing 2Varian Honored Among World's 100 Most Sustainable Corporations 2United Therapeutics Announces Submission Of Pre-Market Approval Application For Implantable Drug Infusion System To Deliver Remodulin 2United Therapeutics Announces Submission Of Pre-Market Approval Application For Implantable Drug Infusion System To Deliver Remodulin 3United Therapeutics Announces Submission Of Pre-Market Approval Application For Implantable Drug Infusion System To Deliver Remodulin 4
... today announced that it has secured $38 million in ... in 2004, the company has raised $92 million in ... round, which consists of two tranches, was led by ... rata participation of existing investors Apax Partners, Clarus Ventures, ...
... April 7 , - ... Ansell Global Healthcare Educational Program, Accepted,for Accreditation , ... leader in healthcare barrier,protective solutions, has announced it has ... community in the area of continued,education. , ...
... Geographical Society,s awards ceremony on Wednesday, April 15, at ... polar explorers - of this planet and of Mars. ... the gargantuan task of relocating the society,s library, described ... the western hemisphere," from New York to Wisconsin in ...
Cached Biology Technology:Aerovance Secures $38 Million in Equity Financing 2Aerovance Secures $38 Million in Equity Financing 3Ansell Healthcare Espresses Firm Commitment to Support Continued Education of Healthcare Professionals Worldwide 2Ansell Healthcare Espresses Firm Commitment to Support Continued Education of Healthcare Professionals Worldwide 3Ansell Healthcare Espresses Firm Commitment to Support Continued Education of Healthcare Professionals Worldwide 4American Geographical Society awards honor Henson, Smith on April 15 at UD 2American Geographical Society awards honor Henson, Smith on April 15 at UD 3American Geographical Society awards honor Henson, Smith on April 15 at UD 4
Mouse monoclonal antibody to PPP2R2C - protein phosphatase 2 (formerly 2A), regulatory subunit B (PR 52), gamma isoform...
These 96-well 0.2 ml thin-wall PCR plate caps are caps that are used to seal 96-well, 0.2 ml thin-wall PCR plates. Supplied as 300 caps....
ImmunoPure Plus Immobilized Protein G, , , , Support: Cross-linked 6%, beaded agarose , Capacity: > 20 mg human IgG/ml of gel ...
Mouse polyclonal antibody to ALS2CR8 - amyotrophic lateral sclerosis 2 (juvenile) chromosome region, candidate 8...
Biology Products: