Article
Evaluation of a transgenic mouse model for alpha-1-antitrypsin (AAT) related liver disease.
Annals of human genetics - 1 Oct 1994
Ali R, Perfumo S, della Rocca C, Amicone L, Pozzi L, McCullagh P, Millward-Sadler H, Edwards Y, Povey S, Tripodi M
Abstract excerpt
We have attempted to produce a transgenic mouse model of the neonatal liver disease associated with the human PIZ allele. Analysis of a number of transgenic mouse lines carrying either a normal human PIM gene construct or the mutant Z is reported. Using isoelectric focusing analysis of plasma from transgenic mice, we have shown that the human AAT proteins produced in mice are processed in a similar way to their...
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