Abstract：
:The production of transgenic mice expressing different forms of the prion protein (PrP) or devoid of PrP has enabled researchers to study the role of PrP in the infectious process of a prion disease and its normal function in the healthy individual. A wide range of transgenic models have been produced ranging from PrP null mice, normal expression levels to overexpression models, models expressing different species of the Prnp gene and different mutations and polymorphisms within the gene. Using this range of transgenic models has allowed us to define the influence of PrP expression on disease susceptibility and transmission, assess zoonotic potential, define strains of human prion diseases, elucidate the function of PrP, and start to unravel the mechanisms involved in chronic neurodegeneration. This chapter focuses mainly on the use of the gene targeted transgenic models and summarizes the ways in which they have allowed us to study the role of PrP in prion disease and the insights they have provided into the mechanisms of neurodegenerative diseases.
journal_name
Prog Mol Biol Transl Scijournal_title
Progress in molecular biology and translational scienceauthors
Diack AB,Alibhai JD,Manson JCdoi
10.1016/bs.pmbts.2017.06.008subject
Has Abstractpub_date
2017-01-01 00:00:00pages
157-179eissn
1877-1173issn
1878-0814pii
S1877-1173(17)30089-3journal_volume
150pub_type
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