Researchers discover cell's 'quality control' mechanismJuly 30, 2008Researchers 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." McGill University | |||||||||||||||||||||
|
Related Quality Control News Articles Clearing the airways in cystic fibrosis By manipulating the machinery used by our cells for quality control, researchers from the University of Pittsburgh have found a way to restore the function of cystic fibrosis (CF) airway cells. Study: DNA barcoding in danger of 'ringing up' wrong species DNA barcoding is a movement to catalog all life on earth by a simple standardized genetic tag, similar to stores labeling products with unique barcodes. The effort promises foolproof food inspection, improved border security, and better defenses against disease-causing insects, among many other applications. Analysis of Lake Washington microbes shows the power of metagenomic approaches Today's powerful sequencing machines can rapidly read the genomes of entire communities of microbes, but the challenge is to extract meaningful information from the jumbled reams of data. Electronic tongue tastes wine variety, vintage You don't need a wine expert to identify a '74 Pinot Noir from Burgundy - a handheld "electronic tongue" devised by European scientists will tell you the grape variety and vintage at the press of a button. NIST trumps the clumps: Making biologic drugs safer Scientists at the National Institute of Standards and Technology (NIST) have developed a technique to measure the formation of clumps of proteins in protein-based pharmaceuticals. Tips on how to build a better home for biological parts Researchers at the Virginia Bioinformatics Institute (VBI) at Virginia Tech have compiled a series of guidelines that should help researchers in their efforts to design, develop and manage next-generation databases of biological parts. RNA emerges from DNA's shadow RNA, the transporter of genetic information within the cell, has emerged from the shadow of DNA to become one of the hottest research areas of molecular biology, with implications for many diseases as well as understanding of evolution. Animal study suggests inadequate sleep may exacerbate cellular aging in the elderly Researchers at the University of Pennsylvania School of Medicine have shown that the unfolded protein response, which is a reaction to stress induced by sleep deprivation, is impaired in the brains of old mice. Ocean warming on the rise Increased scientific confidence that ocean observations are accurately reflecting rising global temperatures is central to new Australian research published today in the journal, Nature. Saltwater Sleuths: NOAA Researchers Seek Clues in Unusual Places to Help Determine the Ages of Fish and Shellfish Populations Fishery biologist Sandy Sutherland looks through the lens of the microscope at tiny sections of fish earbones, known as otoliths, each showing annual bands of growth. More Quality Control News Articles |
|||||||||||||||||||||
|
|||||||||||||||||||||
|
|||||||||||||||||||||