Navigation Links
Researchers discover cell's 'quality control' mechanism

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
McGill University

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:
(Date:4/26/2016)... and LONDON , April 26, ... of EdgeVerve Systems, a product subsidiary of Infosys ... announced a partnership to integrate the Onegini mobile ...      (Logo: ) ... customers enhanced security to access and transact across ...
(Date:4/19/2016)... April 20, 2016 The new ... a compact web-based "all-in-one" system solution for all door ... reader or the door interface with integration authorization management ... control systems. The minimal dimensions of the access control ... the building installations offer considerable freedom of design with ...
(Date:4/15/2016)... , April 15, 2016 ... the,  "Global Gait Biometrics Market 2016-2020,"  report to ... ) , ,The global gait biometrics ... of 13.98% during the period 2016-2020. ... angles, which can be used to compute factors ...
Breaking Biology News(10 mins):
(Date:6/23/2016)... ... June 23, 2016 , ... Supplyframe, ... of the Supplyframe Design Lab . Located in Pasadena, Calif., the Design ... of how hardware projects are designed, built and brought to market. , The ...
(Date:6/23/2016)... BEACH, Calif. , June 23, 2016  Blueprint ... new biological discoveries to the medical community, has closed ... co-founder Matthew Nunez . "We have ... us with the capital we need to meet our ... will essentially provide us the runway to complete validation ...
(Date:6/23/2016)... ... June 23, 2016 , ... Regulatory ... technical consulting, provides a free webinar on Performing Quality Investigations: Getting ... at 12pm CT at no charge. , Incomplete investigations are still a major ...
(Date:6/22/2016)... June 22, 2016 Cell Applications, Inc. ... them to produce up to one billion human ... within one week. These high-quality, consistent stem cells ... cells and spend more time doing meaningful, relevant ... proprietary, high-volume manufacturing process that produces affordable, reliable ...
Breaking Biology Technology: