A PGK-Neo cassette was inserted into exon 5 by homologous recombination to introduce a premature "TAG" stop codon and produce a truncated protein of 416 amino acids with an intact N-terminus but lacking both the poly-Q stretch and following C-terminal peptides. Protein was detected at a level similar to that of the wild-type protein (1,175 amino acids) in brain samples. (J:152709)