A 1.3 kb fragment encompassing exon 2 and flanking intronic sequence was replaced with a neomycin selection cassette inserted by homologous recombination. The deleted region encoded both the DNA-binding and dimerization domains. An aberrant transcript resulting from a splice of exon 1 to exon 3 was identified by RT-PCR analysis of homozygous mutant liver and brain tissues. Sequence analysis indicated that the mutant transcript is out of frame and terminates in exon 3, encoding a polypeptide comprised of 31 residues. (J:81645)