Navigation Links
Pitt researchers describe molecular '2-step' leading to protein clumps of Huntington's disease
Date:3/8/2009

PITTSBURGH, March 8 In a paper published in the early online version of Nature Structural and Molecular Biology, researchers at the University of Pittsburgh School of Medicine deconstruct the first steps in an intricate molecular dance that might lead to the formation of pathogenic protein clumps in Huntington's disease, and possibly other movement-related neurological disorders.

Huntington's is one of 10 diseases in which a certain protein, different for each disease, contains polyglutamine, a stretch of repeating blocks of the amino acid glutamine, explained Ronald Wetzel, Ph.D., professor in the Department of Structural Biology and member of the Pittsburgh Institute for Neurodegenerative Diseases at the University of Pittsburgh School of Medicine. The affected protein in Huntington's disease is called huntingtin.

Most people have a huntingtin protein whose polyglutamine segment contains 20 or so glutamines, and even a polyglutamine with as many as 35 repeats may not cause Huntington's symptoms. But the risk of developing Huntington's disease rises sharply in individuals whose polyglutamine sequences are only slightly larger. A block of 40 repeats, for example, is associated with a very high likelihood of having the disease.

"To a protein chemist, this is a fascinating situation," Dr. Wetzel said. "Polyglutamine doesn't seem to play a sophisticated role in these proteins, and it doesn't have a defined structure. Yet by changing its length to only a very slight extent, it takes on some new physical properties that somehow initiate diseases."

One consequence of the lengthening is protein aggregation, or clumping, a feature that consistently appears in brain cells of patients who have one of these neurodegenerative diseases. Many research groups, including Dr. Wetzel's, study how polyglutamine expansion alters the huntingtin protein's behavior.

In its most recent studies, the Pitt team worked out the details o
'/>"/>

Contact: Anita Srikameswaran
SrikamAV@upmc.edu
412-647-3555
University of Pittsburgh Schools of the Health Sciences
Source:Eurekalert

Page: 1 2

Related biology news :

1. Climate change affecting Europes birds now, say researchers
2. CSHL researchers pinpoint structure-building role for 2 non-coding RNAs
3. Ben-Gurion University of the Negev researchers develop new reversible, green window technology
4. Two UT Southwestern researchers awarded Sloan fellowships
5. 2 McGill researchers garner prestigious NSERC Steacie Fellowships
6. Texas Obesity Research Center at UH assembles researchers to discuss obesity
7. U-M researchers ID gene involved in pancreatic cancer
8. USC researchers identify gene variant associated with both autism and gastrointestinal dysfunction
9. Researchers piggyback to safer reprogrammed stem cells
10. Researchers capture wave of brain activity linked to anticipation
11. UMMS researchers publish DNA identification of czars children
Post Your Comments:
*Name:
*Comment:
*Email:
(Date:7/21/2015)... Today, ZTE announced its Android smartphone Axon, ... expected revenues in 2015 that relate to sales of FPC1025 for ... approximately 2,200 MSEK for 2015. Jörgen Lantto, CEO of ... in China and we are proud that ... for Axon , its first ...
(Date:7/13/2015)... OXFORD, Conn. , Jul. 13, 2015 /PRNewswire/ ... the "Company"), a biometric authentication company focused on ... it has filed provisional patent 62/188684 for ... NXT-ID achieves another new convenient and secure method ... expands new, innovative payment methods, introduced with its ...
(Date:7/8/2015)... 8, 2015 Summary Pancreatic cancer is ... most fatal, with a mortality rate of 10.9 deaths ... pancreatic cancer patients has highlighted a significant need for ... being met by the current market. A highly ... with varying molecule types and mechanisms of action, which ...
Breaking Biology News(10 mins):FPC's Touch Fingerprint Sensor FPC1025 in ZTE's Smartphone Axon 2NXT-ID Patents Behavior-Directed Payments 2NXT-ID Patents Behavior-Directed Payments 3NXT-ID Patents Behavior-Directed Payments 4Frontier Pharma: Pancreatic Cancer - Identifying and Commercializing First-in-Class Innovation 2Frontier Pharma: Pancreatic Cancer - Identifying and Commercializing First-in-Class Innovation 3
... National Cancer Institute (NCI) has awarded Huntsman Cancer Institute investigator ... five years to continue studying the role of a gene ... devastating and sometimes deadly skin cancer. VanBrocklin is an assistant ... Utah. The majority of melanomas have mutations in a ...
... of the fastest-growing open-access scholarly publishers, now part of ... Frontiers in Cell and Developmental Biology journal ... One of the most exciting revolutions occurring in ... in Cell and Developmental Biology will provide a ...
... not want to live a long and healthy life? A ... from ETH Zurich have demonstrated in roundworms. Vitamin B3 ... the worms, diet caused them to live for about one ... of researchers headed by Michael Ristow, a professor of energy ...
Cached Biology News:Niacin, the fountain of youth 2
(Date:7/30/2015)... , July 30, 2015   Aratana Therapeutics, ... therapeutics company focused on the licensing, development and ... today announced that its strategic partner VetStem Biopharma, ... confirmation study of AT-016, an adipose-derived allogeneic stem ... treatment of osteoarthritis pain in dogs. ...
(Date:7/30/2015)... Ascendis Pharma A/S (Nasdaq: ASND ... TransCon technology to address significant unmet medical needs, ... Phase 2 study to evaluate the safety and ... treatment-naïve, pre-pubertal children with growth hormone deficiency, or ... the top-line results from our Phase 2 pediatric ...
(Date:7/30/2015)... ... 2015 , ... As part of its 2015 growth plan and as a follow up to ... business units, Whitehouse Laboratories is pleased to announce that it has begun construction on a ... Excellence and will be strictly dedicated to basic USP 51, USP 61, and USP 62 ...
(Date:7/29/2015)... , July 30, 2015 ... la santé, publie ses résultats pour le premier ... commente les résultats. Visionner l,interview ... http://www.eurobusinessmedia.com/ceo-direct/sanofi/sanofi-h1-2015-results-interview-with-ceo-olivier-brandicourt?utm_source=ceo-direct&utm_medium=wire Au sommaire de ... - Moteurs de croissance - ...
Breaking Biology Technology:Aratana Therapeutics Announces Positive Pilot Field Study for AT-016 2Aratana Therapeutics Announces Positive Pilot Field Study for AT-016 3Aratana Therapeutics Announces Positive Pilot Field Study for AT-016 4Aratana Therapeutics Announces Positive Pilot Field Study for AT-016 5Ascendis Pharma A/S Announces Positive Top-line Results from a Phase 2 Pediatric Study of Once-Weekly TransCon Growth Hormone for the Treatment of Growth Hormone Deficiency 2Ascendis Pharma A/S Announces Positive Top-line Results from a Phase 2 Pediatric Study of Once-Weekly TransCon Growth Hormone for the Treatment of Growth Hormone Deficiency 3Ascendis Pharma A/S Announces Positive Top-line Results from a Phase 2 Pediatric Study of Once-Weekly TransCon Growth Hormone for the Treatment of Growth Hormone Deficiency 4Ascendis Pharma A/S Announces Positive Top-line Results from a Phase 2 Pediatric Study of Once-Weekly TransCon Growth Hormone for the Treatment of Growth Hormone Deficiency 5Ascendis Pharma A/S Announces Positive Top-line Results from a Phase 2 Pediatric Study of Once-Weekly TransCon Growth Hormone for the Treatment of Growth Hormone Deficiency 6Ascendis Pharma A/S Announces Positive Top-line Results from a Phase 2 Pediatric Study of Once-Weekly TransCon Growth Hormone for the Treatment of Growth Hormone Deficiency 7Whitehouse Labs Formally Announces Plans to Open a Microbiological Laboratory 2
... , , , , , Mark Churchill ... , , , , , ... The data presented here can be acquired using , a Thermo Finnigan ... Introduction , , , , , ...
... , , , RohanA.Thakur, AndrewW. Guzzetta, and Julie , ... , Ion Trap Analysis , , , , ... LCQ Advantage, LCQ Deca XP, and Deca XP Plus ion , trap mass spectrometer., ... , , , , , Two important considerations in ...
... , , , , ... Center for Neurologic Diseases, Brigham and Womens Hospital, Harvard , ... , , Genetic manipulations using neuronal cells are notoriously ... , to primary neuronal cultures is of critical importance for ...
Cached Biology Technology:Bioanalytical Method Intraday Validation for the Quantitation of Paroxetine , in Bovine Plasma using the TSQ Quantum Mass Spectrometer 2Bioanalytical Method Intraday Validation for the Quantitation of Paroxetine , in Bovine Plasma using the TSQ Quantum Mass Spectrometer 3Bioanalytical Method Intraday Validation for the Quantitation of Paroxetine , in Bovine Plasma using the TSQ Quantum Mass Spectrometer 4Bioanalytical Method Intraday Validation for the Quantitation of Paroxetine , in Bovine Plasma using the TSQ Quantum Mass Spectrometer 5Bioanalytical Method Intraday Validation for the Quantitation of Paroxetine , in Bovine Plasma using the TSQ Quantum Mass Spectrometer 6Enhancing Ruggedness and Full-Scan MS Sensitivity Using Ion Sweep , Technology 2Enhancing Ruggedness and Full-Scan MS Sensitivity Using Ion Sweep , Technology 3Enhancing Ruggedness and Full-Scan MS Sensitivity Using Ion Sweep , Technology 4Enhancing Ruggedness and Full-Scan MS Sensitivity Using Ion Sweep , Technology 5Enhancing Ruggedness and Full-Scan MS Sensitivity Using Ion Sweep , Technology 6Enhancing Ruggedness and Full-Scan MS Sensitivity Using Ion Sweep , Technology 7Enhancing Ruggedness and Full-Scan MS Sensitivity Using Ion Sweep , Technology 8Efficient RNAi-mediated gene silencing in neuronal cells using QIAGEN , siRNA and TransMessenger Transfection Reagent* 2Efficient RNAi-mediated gene silencing in neuronal cells using QIAGEN , siRNA and TransMessenger Transfection Reagent* 3Efficient RNAi-mediated gene silencing in neuronal cells using QIAGEN , siRNA and TransMessenger Transfection Reagent* 4Efficient RNAi-mediated gene silencing in neuronal cells using QIAGEN , siRNA and TransMessenger Transfection Reagent* 5
... an enzyme that catalyzes the hydrolysis ... maltotriose, and dextrins. The,level of a-amylase ... body is of,clinical significance in the ... diabetes; plant and microbial a-amylases are,important ...
Rat monoclonal [20d5] to NKG2A + C+ E ( Abpromise for all tested applications). Antigen: Tissue / cell preparation (Mouse): CHO transfected cells expressing the B6 allele of NKG2A. Entrez Gen...
... Labsystems Wellmix is a high-quality four-place microplate ... The shaking speed is adjustable from 100 ... speed and a small orbit ensure effective ... time is quick and easy to set ...
... Ascent FL is equipped ... luminometric measurement technologies that ... of advanced features of ... Ascent. Fluoroskan Ascent FL ...
Biology Products: