Navigation Links
Scripps Research scientists solve mystery of nerve disease genes
Date:7/4/2011

obtain the structures of five different GlyRS mutants and the wild-type protein in solution. This method, called hydrogendeuterium exchange, gives information about which parts of a protein are in touch with a solutionin other words, which parts lie on the outside rather than being buried deep inside its three-dimensional shape. In collaboration with Min Guo, assistant professor at Scripps Florida, a second method, called small angle X-ray scattering, was used to measure changes in the overall shape of the protein structure in solution.

Together, the results revealed that every GARS mutation studied causes a structural opening in the resulting mutant protein. By superimposing the information obtained from the structures in solution to the high-resolution X-ray crystal structure of the wild-type GlyRS protein, Yang, He, Zhang and colleagues were able to determine that the structural opening maps to a common region of the GlyRS protein in all the mutants.

By becoming more open to potential new partners, the GlyRS mutants may gain a new function that is toxic to nerve cells. That would explain why CMT type 2D is inherited in a dominant fashion.

Exciting Implications

The team is now looking for proteins that might bind to this region and already have leads on several candidates. As exciting as this work is, says He, "this paper is just the start of the story; we have more story coming, and it's getting more and more exciting."

This disease mechanism may also apply to other conditions. "Some proteins may be relatively unstable and can be easily triggered into another conformation by different types of mutations," explained Yang. "This example deals with the GlyRS protein, but the general idea can be applied to many other mutation-induced human diseases."

One example may be ALS. Some inherited forms of ALS are caused in a gene that encodes the enzyme copper-zinc superoxide dismutase (commonly called SOD1). To da
'/>"/>

Contact: Mika Ono
mikaono@scripps.edu
858-784-2052
Scripps Research Institute
Source:Eurekalert

Page: 1 2 3 4

Related biology news :

1. Scripps awarded $7.9 million NIH grant for research to find root cause of heart attack
2. Scripps study finds plastic in 9 percent of garbage patch fishes
3. Scripps Research Institute Scientist Knighted by Italian Republic
4. Scripps Research team sheds new light on how blood clots form
5. Scripps Research scientists awarded $2.2 million to develop treatment for multi-drug addiction
6. Scripps Research scientist wins $1.9 million grant to study malaria
7. Scripps Research scientists find way to block stress-related cell death
8. Scripps Research scientists find key mechanism in transition to alcohol dependence
9. Scripps Research scientists create new genetic model of premature aging diseases
10. Scripps Research scientists identify mechanism of long-term memory
11. Scripps Research scientists uncover new DNA role in modifying gene function
Post Your Comments:
*Name:
*Comment:
*Email:
(Date:8/20/2014)... honeybees grows to about 4,000 members, it triggers an ... of a special type of comb used for rearing ... the Department of Neurobiology and Behaviour at Cornell University, ... cycle of honeybee colonies. The results are published in ... . , Reproduction isn,t always a honeybee colony,s ...
(Date:8/19/2014)... decrease China,s increasing caesarean section rate, suggests a new ... International Journal of Obstetrics and Gynaecology (BJOG) . , ... in the world. Of 16 million babies born in ... rate is not known, the current Chinese language literature ... ranging from 36% to 58%. However, before the 1980s, ...
(Date:8/19/2014)... It,s not a monkey. It,s not a lemur. It,s ... Meet the Philippine tarsier: a tiny, adorable and downright "cool" ... animals that Americans are familiar with," said Rafe Brown, curator-in-charge ... giant eyes and ears; an extremely cute, furry body; a ... interesting expanded fingers and toe tips that look a bit ...
Breaking Biology News(10 mins):Worker bees 'know' when to invest in their reproductive future 2Is China's 50 percent cesarean section delivery rate too high? 2Is China's 50 percent cesarean section delivery rate too high? 3Philippine tarsier gets boost from Kansas research, and genetic proof of a new variety 2Philippine tarsier gets boost from Kansas research, and genetic proof of a new variety 3
... University may make a difference one day at the gas ... organic compound on earth, has enough energy to be the ... break it down could be devised. Cellulose is a cell ... assistant professor in Kansas State University,s Division of Biology, has ...
... available in Spanish . Hake is ... and, nevertheless, resists stock depletion relatively well. At times nature ... Nerea Goikoetxea, a researcher at Azti-Tecnalia, has been witness to ... European hake population, observing which environment has favoured the species ...
... While highly potent embryonic stem cells are often the subject of ... we age, our stem cells are less pliant and less able ... breakthrough treatments for disease. An exception to this can be ... lines the inside of our mouths. These cells do not seem ...
Cached Biology News:Learning secrets of world's most common organic compound driving research for biofuels 2Hake population has withstood overfishing, thanks to the warming of the sea 2Hake population has withstood overfishing, thanks to the warming of the sea 3'Open wide' for new stem cell potential 2'Open wide' for new stem cell potential 3
(Date:8/21/2014)... St. Louis, MO (PRWEB) August 21, 2014 ... premier, full service biopharmaceutical contract development and manufacturing ... selected by Progenics Pharmaceuticals, Inc., an oncology company ... targeting and treating cancer, to manufacture the anti-prostate ... Progenics’ PSMA ADC product candidate. Under the ...
(Date:8/20/2014)... inventions licensed to biotechnology firms has revealed early ... these roadblocks, the researchers suggest that better communication ... could lead to faster commercialization down the road. ... made in university laboratories and licensed to biotechnology ... which have a high failure rate. But a ...
(Date:8/20/2014)... 20, 2014 High performance computing ... the most complete climate and Earth system model ... change issues. , Eight national laboratories, including ... National Center for Atmospheric Research, four academic institutions ... Other participating national laboratories include Argonne, Brookhaven, Lawrence ...
(Date:8/20/2014)... Not long ago, it was more or ... late in the development cycle – even after the ... suggest that, like many things GxP, expectations for early ... multiple drug sponsors have been required by authorities to ... phase I/II. It’s not only clinical trial material containers ...
Breaking Biology Technology:Gallus Enters Agreement with Progenics Pharmaceuticals, Inc. for Manufacture of Anti-PSMA Monoclonal Antibody 2Gallus Enters Agreement with Progenics Pharmaceuticals, Inc. for Manufacture of Anti-PSMA Monoclonal Antibody 3Early bottlenecks in developing biopharmaceutical products delay commercialization 2Early bottlenecks in developing biopharmaceutical products delay commercialization 3New Project Is the ACME of Addressing Climate Change 2New Project Is the ACME of Addressing Climate Change 3Are Extractable and Leachables going Phase-Appropriate? New Webinar Hosted by Xtalks 2
... Sept. 16 Martek Biosciences (Nasdaq: MATK ) today ... Directors. , , Mr. Pernock brings ... products, having served from 1994 to 2009 in a variety of ... companies. In his most recent executive role, Mr. Pernock served as ...
... , HEIDELBERG, Germany, September 16 ... Proteomics Approach Identifies new,Potential Drug Targets in Wnt Pathway , ... "Tankyrase inhibition,stabilizes axin and antagonizes Wnt signalling"** is now available ... how Cellzome,s quantitative chemical proteomics,platform was used to identify a ...
... , , SAN ... ) announced today that Kevin Gorman, President and Chief Executive Officer ... Sciences Conference in New York. , , ... p.m. Eastern Time (ET)/10:00 a.m. Pacific Time (PT). The presentation ...
Cached Biology Technology:Martek Announces Addition to Its Board of Directors 2Cellzome Technology Used to Unravel Novel Regulatory Mechanism in Cancer Signaling Pathway 2
cDNA Size Fractionation Columns offer an efficient and convenient method for size-fractioning double-stranded cDNA....
... delivery of buffer and fixative results ... version. In Vivo Manual Perfusion Fixation ... of buffer and fixative results in ... use and clean--Complete system with everything ...
... your 35mm dish without transferring to ... has separate openings for solution inflow ... of the liquid level in the ... entering the chamber. An adjustable metal ...
Sf21 cells derived from Spodoptera frugiperda ovarian cells are recommended for high-level production of recombinant proteins. Higher expression levels of some proteins are seen due to the larger ce...
Biology Products: