A mouse cDNA encoding paired-box-containing gene (Pax-4) was cloned from a cDNA library of a mouse pancreatic islet beta cell line of MIN6. The predicted open reading frame encodes a protein of 349 amino acids with a calculated molecular weight (MW) of 38-kD. A human Pax-4 cDNA sequence encoding 350 amino acids was deduced from a human cosmid clone. The mouse nucleotide and deduced amino acid sequences exhibit 83.4 and 80.0% identity with those of deduced human Pax-4, respectively. Southern blot analysis suggested that the mouse Pax-4 gene exists as a single copy in the genome. Reverse transcription (RT)-PCR analysis suggested that the mouse Pax-4 gene is expressed in pancreatic islets, cultured islet beta cell lines of MIN6, beta TC, and NIT-1 cells, but not detectable in any of 13 adult mouse organs examined.