SHH and progressive pseudorheumatoid arthropathy of childhood: Further, cis-regulatory analyses identified GLI-consensus DNA binding sites and SHH-GLI3 regulation of Pax9, Jag1, and Hes1[17,18], however it is unknown whether any of these genes are directly responsible for SHH-GLI3 function in regulating digit number during normal autopod development or leading to the PPD pathology.