PHARMACEUTICAL COMPOSITION FOR THE PREVENTION OR TREATMENT OF A PRION DISEASE CONTAINING AN ACTIVE INGREDIENT IN THE FORM OF THE PRNP GENE USING RECOMBINANT ADENOVIRUS VECTOR

摘要

The present invention relates to a pharmaceutical composition for the prevention or treatment of a prion disease containing an active ingredient in the form of the Prnp gene using a recombinant adenovirus vector. In a system in which the Prnp gene is transfected into a mouse nerve cell strain (Zpl 3-4) from which the Prnp gene has been knocked out by using the recombinant adenovirus vector according to the present invention, in the normoxic state PrPC is over-expressed, and the proportion of annexin V (+) cells, which is to say cells where apoptosis has occurred, and the LDH activity are significantly reduced as compared with Zpl 3-4 cells transfected with a recombinant adenovirus vector (Ad-lacZ) having no expression cassette. Consequently, The Prnp gene transfection system using a recombinant adenovirus vector according to the present invention protects nerve cells by suppressing PrPSc-mediated nerve cell death, and thus can be used to advantage in the prevention or treatment of prion diseases.