Prion diseases related peptides

Prion diseases or transmissible spongiformencephalopathies (TSEs) are a family of rare progressive neurodegenerativedisorders that affect both humans and animals. They are distinguished by longincubation periods, characteristic spongiform changes associated with neuronalloss, and a failure to induce inflammatory response.

The causative agents of TSEs are believed to be prions.The term "prions" refers to abnormal, pathogenic agents that aretransmissible and are able to induce abnormal folding of specific normalcellular proteins called prion proteins that are found most abundantly in thebrain. The functions of these normal prion proteins are still not completelyunderstood. The abnormal folding of the prion proteins leads to brain damageand the characteristic signs and symptoms of the disease. Prion diseases areusually rapidly progressive and always fatal.

Familial prion diseases of humans include classicCreutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker syndrome (GSS),and fatal insomnia (FI). These conditions form a spectrum of diseases withoverlapping signs and symptoms.The prevalent controversial ‘protein-only’ hypothesisproposes that the infectious agent is a misfolded protein that triggers andpropagates the disease in the absence of nucleic acid. The prion protein is thecause for all known mammalian prion diseases. Prion proteins are thought toexist in two different conformations. A properly folded form is denoted PrP^C(for Common or Cellular)and a misfolded form is denoted PrP^Sc (for Scrapie,after one of the diseases first linked to prions and neurodegeneration.) Theprecise structure of the prion is not known but it can be formed by combiningPrP^C, polyadenylic acid, and lipids in a Protein Misfolding CyclicAmplification (PMCA) reaction.

The protein encoded by the major prion protein gene is amembrane glycosylphosphatidylinositol-anchored glycoprotein that tends toaggregate into rod-like structures. The encoded protein contains a highlyunstable region of five tandem octapeptide repeats. This gene is found onchromosome 20, approximately 20 kbp upstream of a gene which encodes abiochemically and structurally similar protein to the one encoded by this gene.Mutations in the repeat region as well as elsewhere in this gene have beenassociated with Creutzfeldt-Jakob disease, fatal familial insomnia,Gerstmann-Straussler disease, Huntington disease-like 1, and kuru. Alternativesplicing results in multiple transcript variants encoding the same protein.

Endotoxin Highlight

Lipopolysaccharides (LPS), commonly known as endotoxin are found in Gram-negative bacteria outer membrane.  Much evidence has shown the ability of LPS in eliciting immune response in human and animalsincluding shock, coagulation, fever and inflammation.  At high concentration, LPS evoke a massive circulation of macrophages and cause major clinical issue in septic shock treatment in human.  Despite the complication that LPS can cause in human, LPS also post a major concern in animal research due to the ability of LPS in inducing immune response. During animal immunization, an antigen is introduced for specific antibody production against the antigen.

  However, the immune response is manifested with the presence of LPS and it often lead to the antibody production against LPS instead of the antigen introduced.  Gram-negative bacteria such as E-coligain much attention in bioengineering and microbiology research. E-coliare best known as the versatile host for protein expression, recombinant protein production and being a vector.  Though, there are many benefits for using E-coliin research, the LPS that are in conjunction could affect the research accuracy and specificity.  For that reason, LPS removal is crucial for all kind of animal research especially when it is related to gram-negative bacteria. 

Bio-Synthesis provides rapid endotoxin detection that is approved.  Despite the endotoxin detection service, we also offer customized endotoxin removal service for our customers.  Our methods offer up to a 90% efficiency of endotoxin removal and with up to 80% protein recovery.