Navigation Links
Researchers discover cell's 'quality control' mechanism
Date:7/29/2008

This release is available in French.

Researchers in Japan and Canada have discovered a key component of the quality control mechanism that operates inside human cells sometimes too well. The breakthrough has significant implications for the development of new treatments for cystic fibrosis (CF) and some other hereditary diseases, the researchers say. Their results were published July 25 in the journal Science.

Dr. Kazahiro Nagata and colleagues at Kyoto University and the Japan Science Technology Agency, and Dr. David Thomas and Dr. Gregor Jansen at McGill University in Montreal, have discovered the important role played by an enzyme called ERdj5 inside the cell's endoplasmic reticulum (ER). The ER acts as a sort of packaging plant that folds and prepares proteins for distribution inside or outside the cell. But when proteins are misfolded in the ER, they must be destroyed in a degradation process and that is where ERdj5 comes into play.

"ERdj5 is like a quality control inspector," explained Dr. Thomas, McGill's Chair of Biochemistry and Canada Research Chair in Molecular Genetics. "If you ever owned an AMC Pacer and you now drive a BMW, you know the difference quality control can make. That's what ERdj5 does, it recognizes when a protein has 'manufacturing defects' and degrades it before it can be distributed."

The ERdj5 enzyme is the first protein found to be capable of breaking the disulfide bonds that hold the misfolded proteins together in the ER. Once those bonds are broken, the researchers say ERdj5 also helps other enzymes and molecules break down the misfolded proteins completely so that the constituent amino acids can be recycled for further protein synthesis.

"Unfortunately, the mechanism sometimes works a little too well," Dr. Thomas said. "It insists on BMW quality when a Honda would do. For example, some people carry a mutated version of the protein CFTR. The mutated protein is damaged but would still work fine if it were distributed, but in some individuals, the quality control mechanism insists on degrading it. It's the degradation of the protein, not the mutation itself, which causes cystic fibrosis. We're hoping this discovery will open up new avenues of research into treatments for CF."


'/>"/>

Contact: Mark Shainblum
mark.shainblum@mcgill.ca
514-398-2189
McGill University
Source:Eurekalert

Related biology news :

1. Researchers analyze how new anti-MRSA abtibiotics function
2. Researchers tap into a new and potentially better source of platelets for transfusion
3. CMV infections affect more than just patients with compromised immune systems, researchers find
4. A new cellular pathway linked to cancer is identified by NYU researchers
5. Researchers find key to saving the worlds lakes
6. MIT researchers offer glimpse of rare mutant cells
7. UC San Diego researchers could help US military thwart explosive threats
8. As rates rise, researchers find better way to identify melanoma
9. Researchers discover a gene that regulates and blocks ovulation
10. Researchers discover link between organ transplantation and increased cancer risk
11. Dartmouth researchers discover gene signatures for scleroderma
Post Your Comments:
*Name:
*Comment:
*Email:
(Date:4/26/2016)... and LONDON , April ... part of EdgeVerve Systems, a product subsidiary of ... today announced a partnership to integrate the Onegini ...      (Logo: http://photos.prnewswire.com/prnh/20151104/283829LOGO ) ... their customers enhanced security to access and transact ...
(Date:4/19/2016)... DUBAI , UAE, April 20, 2016 ... can be implemented as a compact web-based "all-in-one" system ... in the biometric fingerprint reader or the door interface ... requirements of modern access control systems. The minimal dimensions ... the ID readers into the building installations offer considerable ...
(Date:4/15/2016)... Research and Markets has announced ... 2016-2020,"  report to their offering.  , ... global gait biometrics market is expected to grow ... 2016-2020. Gait analysis generates multiple variables ... to compute factors that are not or cannot ...
Breaking Biology News(10 mins):
(Date:4/29/2016)... San Diego, CA (PRWEB) , ... April 29, 2016 , ... ... Cell Foundation (NSCF) to support the development of a patient-specific stem cell therapy for ... Bratt-Leal in the lab of Dr. Jeanne Loring at The Scripps Research Institute in ...
(Date:4/28/2016)... ... April 28, 2016 , ... Connecticut Innovations ... growing companies, today announced the launch of VentureClash , a $5 million ... , “VentureClash looks to attract the best early-stage companies here in Connecticut, ...
(Date:4/27/2016)... ... 2016 , ... Shimadzu Scientific Instruments (SSI) will be showcasing ... Conference and Expo. Shimadzu’s high-performance instruments enable laboratories to test cannabis products for ... stop by booth 1021 to learn how Shimadzu’s instruments can help improve QA/QC ...
(Date:4/27/2016)... ... April 27, 2016 , ... ... to announce the appointment of John Tilton as Chief Commercial Officer.  Mr. Tilton ... one of the founding commercial leaders responsible for the commercialization of multiple orphan ...
Breaking Biology Technology: