Focus on progress! The first all-human mouse model of hereditary prion disease opened successfully
Human viral diseases include Creutzfeldt-Jakob disease (CJD) and Gerstmann syndrome (GSS). Scientists from University College London and other institutions recently published a research report in the international journal PLoSBiology, which is based on the results of studies on mouse models of human viruses and the infectivity of related viruses. We found that the spontaneous formation of viral protein assemblies can only occur in mice carrying human viral proteins. Pri virus disease is caused by the misfolding of ion virus protein and cell-to-cell communication, which in turn causes misfolding of recipient cells. A distinguishing feature of the disease is that the diseases caused by different mutations are obviously different; clinical manifestations: when studying these diseases, in order to understand the prevalence and pathological mechanisms of the virus disease, various diseases should be studied. Analysis of specific strains is crucial. By introducing genes that mainly encode viral proteins and carry pathogenic mutations, Prion disease can be simulated in mice. In previous studies, researchers did not directly make pathogenic mutations in human viral protein genes; instead, they introduced equivalent genes into mouse viral protein genes, which resulted in the formation and spread of unfolded proteins. . This limits scientists' understanding of human viral diseases. To overcome this problem, the researchers introduced the mutant virus gene into mice without the mouse the virus gene. When mice grow ion virus protein clusters for more than a year and a half, misfolding naturally occurs, which researchers have not observed before; when these protein clusters are transferred to young mice with the same mutation, they It is wrong. It can also simulate the infectivity of the patient, which can explain the mutant protein that produces the folded protein cluster and the same mutant egg in the patient.
White clusters, this is also the first time that researchers have discovered spontaneous infections entirely caused by mutated human prion proteins in mice.
Finally, the researcher Asante said that this new genetic prion disease model may help us understand the pathogenesis of human prion diseases, which could not be obtained through research on mice before, including studying the occurrence of diseases. Incidents, spread, and the development of new therapies for later prion diseases.
小鼠模型,动物造模
2020-07-23
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