desert Hedgehog (DHH) (AA 23-198), (Cys23IleIle-Mutant) (Active) protein
Description
Desert hedgehog protein (DHH) is a member of the Hedgehog family which encodes signaling molecules that play an important role in regulating morphogenesis. It is predicted to be made as a precursor that is auto-catalytically cleaved, the N-terminal portion is soluble and contains the signaling activity while the C-terminal portion is involved in precursor processing. More importantly, the C-terminal product covalently attaches a cholesterol moiety to the N-terminal product, restricting the N-terminal product to the cell surface and preventing it from freely diffusing throughout the organism. Defects in this protein have been associated with partial gonadal dysgenesis (PGD) accompanied by minifascicular polyneuropathy. DHH may be involved in both male gonadal differentiation and perineurial development. DHH binds both Patched and Patched 2 as well as Hedgehog interacting protein (Hip). It induces steroidogenic factor 1(SF1), which is instrumental in promoting Leydig cell differentiation. It also promotes the deposition of basal lamina surrounding seminiferous tubules. Recombinant human Desert hedgehog (DHH) produced in Escherichia Coli is a single non-glycosylated polypeptide chain containing 177 amino acids. A fully biologically active molecule, rhDHH has a molecular mass of around 20kDa analyzed by reducing SDS-PAGE. Synonyms: DHH, Human