We have isolated the promoter region and part of the coding region of the human plasminogen activator inhibitor 2 gene. The first two exons (65 and 177 base pairs, respectively) are separated by a 3.6 kilo base pair intron. More than 2 kilo base pair of the promoter region was sequenced and analyzed. The promoter region is characterized by: a) a TATAAA box located 31 base pairs upstream of the transcription initiation site; b) a 123 base pair stretch (-598 to -720) that is homologous (greater than 90% identity) to the inverted sequence of -1268 to -1380; c) a 24 base pair inverted repeat at -276 to -299 and three direct repeats; and d) several sequences that are homologous (up to 1 mismatch) to the cAMP responsive element or to binding sites for the transcription factors AP1 and AP2.