A sick cow may also act very nervous or violent, which is why BSE is often called mad cow disease. The disease belongs to the group of transmissible spongiform encephalopathies, which also includes scrapie of sheep and goats and chronic wasting disease (CWD) of elk and deer. A sick cow has trouble walking and getting up. Three major trials genetic manipulation with dominant negative mutant PrP C gene working against a hypothetical host-specific factor, antibody therapy with anti-PrP antibodies which block PrP C-PrP Sc binding, and PrP Sc unfolding therapy with a novel-class molecular chaperone, are currently underway. Abnormal prions are a special type of pathogen that has no genes and is the infectious agent in diseases such as bovine spongiform encephalitis (BSE/mad cow. Bovine spongiform encephalopathy (BSE), sometimes referred to as 'mad cow disease', is a chronic degenerative disease affecting the central nervous system of cattle. These studies also help us to develop "therapeutics and prevention methods" for prion disease.
Our prion research focuses on further understanding such an unprecedented mechanism by identifying auxiliary factor(s) other than PrP C and PrP Sc. Protein chaperones act to promote folding, block aggregation, disaggregate proteins, and facilitate protein degradation ( Duncan et al., 2015 Finka et al., 2015 ). The amino acid sequences of PrP C and PrP Sc are identical, but their conformations are rather different PrP C is rich in the non β-sheet isoform while PrP Sc is rich in the β-sheet isoform. Great concern about variant CJD, which is caused by ingesting BSE-contaminated products, is also emerging and spreading over Japan since the first BSE-affected cattle was identified in September, 2001. Small-molecule pharmacological chaperones can inhibit propagated.
Prion protein (PrP) exists in two different isoforms a normal cellular isoform (PrP C) and an abnormal infectious isoform (PrP Sc), the latter is a causative agent of prion disease such as Bovine Spongiform Encephalopathy (BSE, mad cow disease) and Creutzfeldt-Jakob disease (CJD). Prion and prion-like diseases involve the propagation of misfolded protein conformers. diseases such as sickle cell anemia, mad cow disease, and Alzheimers disease are caused by unnatural aggregation of proteins into cell-clogging fibrils.
0 kommentar(er)
