The present invention is related with the neurobiology and the transgenic
animals, more specifically with obtaining a model to study the SCA2
genetic disease. The genome of one mice line has been modified through
the introduction of a DNA segment that contains the necessary information
for the synthesis of the human ataxin 2. In particular the invention is
related with the transgenic mice F066 and the cellular lines able to
express the human sca2 gene under the regulation of its self promoter.
The F066 transgenic mice reproduce the features of the disease. The
homozygous transgenic mouse line for the new gene was obtained through
matting. This transgenic line is useful to study the mechanism that
produce the disease and also will permit to prove new therapeutics that
contributes to minimize the clinical symptoms in the patients.