Transgenic studies spontaneous disease

In recent years studies have been developed in spontaneous disease models, gene knock out models and transgenic animals. These models provide information on the pharmacological action, pharmaco-kinetics and tolerability of a biotech products. 

The caveat with all of the current transgenic mouse models of familial TSE is that none precisely replicate the human disease. Almost all of them are dependent on transgenic mouse studies that involve both overexpression and random insertion of the transgene. In fact, when a single copy of a PrP mutant associated with GSS (proline to leucine at 102) is specifically substituted into the PrP gene locus, no spontaneous neurodegenerative disease is observed (Manson et al, 1999). Rather, an increased resistance to infection with scrapie is found (Manson et al,... 

There remains considerable controversy as to whether copper actually accelerates the onset of AD. Two studies demonstrated that copper appeared to lower the amyloid burden in the AD brain of APP transgenic mice. In the first study Phinney et al., 2003 demonstrated an in vivo reduction of amyloid-A by a mutant copper transporter [134]. They used a well-known spontaneous mutation of a special strain of mutant toxic-milk mice that accumulate too much copper. Eventually those animals get a liver disease which is a facsimile of a human disorder called Wilson s disease. In direct contrast to the results of Sparks and Scheur (2003) [30], this study demonstrated that an excess of copper in the brain actually reduced of amyloid burden. 

---