A floxed neo cassette was inserted into intron 3 and exon 4 was replaced with one which contained codons for 6 altered amino acids followed by a premature stop codon as is seen in a human frameshift mutation. Transient cre expression in ES cells was used to remove the neo cassette. Western blot analysis of skeletal muscle confirmed the expression of a truncated protein consisting of 634 amino acid residues. (J:134157)