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:1/27/2016)... , Jan. 27, 2016  Rite Track, Inc. a ... West Chester, Ohio announced today ... service staff, based in Austin, Texas ... ability to provide modifications, installations and technical support offerings ... CEO of PLUS, commented, "PLUS has provided world class ...
(Date:1/21/2016)... , January 21, 2016 ... a new market research report "Emotion Detection and Recognition ... Software Tools (Facial Expression, Voice Recognition and Others), ... Global forecast to 2020", published by MarketsandMarkets, the ... to reach USD 22.65 Billion by 2020, at ...
(Date:1/15/2016)... Puerto Rico , Jan. 15, 2016 ... big and small to find new ways to ensure ... culture. iOS and Android ... based on biometrics, transforming it into a hardware authorization ... that users swipe their fingerprint on their KodeKey enabled ...
Breaking Biology News(10 mins):
(Date:2/11/2016)... ... February 11, 2016 , ... ... a new agreement with Bankok,Thailand-based Global Stem Cells Network (GSCN) to distribute exosome ... Latin American countries, including Mexico, Costa Rica, Dominican Republic, Colombia, Argentina, Nicaragua, Panama, ...
(Date:2/10/2016)... - BioAmber Inc. (NYSE: BIOA ), a leader ... & Co. Ltd., its partner in the ... an additional CDN$25 million in the joint venture for ... to 40%.  Mitsui will also play a stronger role ... Sarnia , providing dedicated resources alongside BioAmber,s ...
(Date:2/10/2016)... , Feb. 10, 2016  The Maryland House ... , has announced that University of Maryland School of ... MBA and University of Maryland Medical System President and ... the "Speaker,s Medallion," the highest honor given to the ... Delegates. Dean Reece and Mr. Chrencik ...
(Date:2/10/2016)... 2016 ASAE is introducing a hybrid membership ... (AMC) the option of joining or renewing through an ... by staff size, every employee in any size association ... reap all available member benefits.   John ... membership options will allow organizations of any size and ...
Breaking Biology Technology: