The mouse genome contains at least three copies of sequences homologous to the "paired box", a conserved domain in several Drosophila segmentation genes of the pair-rule and segment polarity classes. Overlapping phages were isolated from two different genomic libraries using the Drosophila gooseberry distal paired box as a probe. The hybridizing sequences are highly homologous to the conserved Drosophila paired box sequences. A single 3.1 kb Pax 1 (paired box gene) transcript was detected during embryonic development, whereas no transcripts were detected in adult tissues. Detailed in situ hybridization analyses with frozen embryonic sections demonstrated Pax 1 transcripts in the perichordal zone of the developing vertebral column. The expression pattern suggests a role for this gene in the formation of segmented structures of the mouse embryo.