Mouse models of this disease include R6/2 and BACHD, which were used to test the V(L)12.3intrabody to treat it.
Mouse models of this disease include R6/2 and BACHD, which were used to test the V(L)12.3intrabody to treat it. A group of diseases similar to this one are caused by mutations injunctophilin-3. The mechanism of this disorder is similar to the one that causes Kennedy disease orMachado-Joseph disease. The affected protein in this disease is SUMOylated by Rhes, which ismainly expressed in the striatum. This disease shows anticipation, in which successive (*) generationsare affected at an earlier age due to further expansion of a poly-glutamine tract. Symptoms of this diseaseinclude the inability to swallow and uncoordinated, dancelike movements called chorea. For 10 points,name this autosomal dominant neurological disease named for an American physician.
Mouse models of this disease include R6/2 and BACHD, which were used to test the V(L)12.3intrabody to treat it.
Huntington's disease