The homeobox gene Nkx-3.1 is the human homolog of Drosophila bagpipe, which, in conjunction with tinman, determines cell fate in the dorsal mesoderm. In mammalian species, Nkx-3.1 is predominantly expressed in prostate, and it regulates prostate development in response to sonic hedgehog (Shh) signaling by exerting growth-suppressive and differentiating effects on prostatic epithelium. Nkx-3.1 is also expressed at lower levels in other tissues, including the heart and gut, in a Shh independent manner, where it plays a role in regulating proliferation of glandular epithelium and in the formation of ducts in prostate and minor salivary glands. Nkx-3.1 preferentially binds the TAAGTA sequence, which has not been reported for any other NK class homeoprotein. The human Nkx-3.1 gene is located on chromosome 8q21, which frequently undergoes a loss of heterozygosity, and although Nkx-3.1 is not a tumor suppressor gene, it may be a useful marker for benign and malignant prostate epithelium.