HUNTINGTON DISEASE CELLULAR MODEL: STABLY TRANSFECTED PC12 CELLS EXPRESSING MUTANT HUNTINGTIN

摘要

The invention provides compositions and methods for the study, diagnosis, and treatment of Huntington's disease. Compositions comprise stably transfected cell lines that serve as a cellular model for Huntington's disease, sequences corresponding to an antigen associated with Huntington's disease, and monoclonal antibodies that bind specifically to said antigen. The stably transfected cell lines of the invention express truncated mutant huntingtin protein and display cellular defects similar to those observed in cells of patients with Huntington's disease. A method is provided wherein these cell lines are used to screen for therapeutic agents for the treatment of Huntington's disease. The monoclonal antibodies of the invention find use in a diagnostic assay for Huntington's disease.