Synonyms
DHH; HHG-3MGC35145
Description
Desert Hedgehog (Dhh) belongs to the highly conserved Hedgehog family of proteins which are involved in multiple developmental processes. Desert Hedgehog is a secreted, 45 kDa, 373 amino acid (aa) protein that undergoes autocatalytic cleavage between Gly198 and Cys199 catalyzed by the C-terminal domain, which releases the N-terminal domain with a concominant attachment of cholesterol at its new C-terminus. In addition to the C-terminally attached cholesterol, a fatty acid acyl chain is esterified to the N-terminal cysteine (aa 23) via an amide linkage. The 19 kDa N-terminal signaling domain is membrane associated due to its double lipid modifications. Its binding to Patched receptors results in the loss of Patched repression of Smoothened signaling. Dhh binds both Patched and Patched 2 as well as Hedgehog interacting protein (Hip). Within the N-terminal domain, human Dhh shares 97% aa sequence identity with mouse and rat Dhh. It shares approximately 75% aa seqeuence identity with human Indian (Ihh) and Sonic Hedgehog (Shh). Dhh is produced by Sertoli cells and is required for testis development and spermatogenesis. It induces steroidogenic factor 1, which is instrumental in promoting Leydig cell differentiation. It also promotes the deposition of basal lamina surrounding seminiferous tubules. Mutations of Dhh are linked to complete pure gonadal dysgenesis. Dhh is expressed in the female by ovarian granulosa cells and the corporus luteum. Its upregulation in ovarian cancer correlates positively with proliferative index, and negatively with prognosis. Dhh is also expressed by Schwann cells and is upregulated following nerve injury. It induces the expression of Patched and Hip in neural tissue fibroblasts and promotes the formation of the connective tissue sheath surrounding peripheral nerves.
Molecular Weight
Approximately 19.9 kDa.
AA sequence
IIGPGRGPVG RRRYARKQLV PLLYKQFVPG VPERTLGASG PAEGRVARGS ERFRDLVPNY NPDIIFKDEE NSGADRLMTE RCKERVNALA IAVMNMWPGV RLRVTEGWDE DGHHAQDSLH YEGRALDITT SDRDRNKYGL LARLAVEAGF DWVYYESRNH VHVSVKADNS LAVRAGG
Appearance
Sterile Filtered White lyophilized (freeze-dried) powder.
Purity
> 96% by SDS-PAGE and HPLC analyses.
Biological Activity
The ED50 as determined by its ability to induce alkaline phosphatase production by C3H10T1/2 (CCL-226) cells is 15-45 μg/mL. Fully biologically active when compared to standard.
Endotoxin
< 1.0 EU per 1μg of the protein by the LAL method.
Formulation
Lyophilized from a 0.2 μm filtered concentrated solution in 10mM PB, pH 6.0, 300mM NaCl.
Reconstitution
We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Reconstitute in sterile distilled water or aqueous buffer containing 0.1% BSA to a concentration of 0.1-1.0 mg/mL. Stock solutions should be apportioned into working aliquots and stored at ≤ -20℃. Further dilutions should be made in appropriate buffered solutions.