Canada at McGill University and the Japan Kyoto University scientists in the human body cells found a way to engage in quality control of ERdj5, but sometimes the enzymes work too seriously, has become the main cause of certain genetic diseases. Researchers believe that the find help for some genetic diseases like cystic fibrosis (hereditary diseases of the pancreas) to find new treatments. Related study, published in the July 25 issue of the journal Science.
researchers found in the endoplasmic reticulum of the cell this enzyme called ERdj5. Role of endoplasmic reticulum like packaging plant cells, protein packed ready for the orderly distribution of proteins in the cell or cells in vitro. However, if the proteins in the ER, packaging errors occur, the defective protein must be in a degradation in the process of being destroyed. ERdj5 enzymes are playing a role in the degradation process.
ERdj5 enzyme found so far is the first has to break the protein of the disulfide bond, and it is the disulfide defective protein in the endoplasmic reticulum to get together. Involved in the study of molecular genetics at McGill University experts said Dr Thomas, ERdj5 enzyme’s function as a quality inspector, be able to identify the defective proteins and degrade these proteins for posts before it.
is, unfortunately, such enzymes work too seriously sometimes, too strict on protein quality. For example,. some people carry a Variant CFTR protein, although these variations of protein damage, but can still be competent for the job after the induction. However, overly strict quality inspector still insists to degrade it. Due to the degradation of proteins, rather than the variations itself, thereby causing cystic fibrosis (hereditary diseases of the pancreas). Based on this, the researchers believe, that for the treatment of the genetic disease discovered found a new avenues of research.
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