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DHH

     
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Product Information
Primary Accession O43323
Species Human
Sequence Cys23Gly198 (Cys23Ile)
Purity > 95% as analyzed by SDS-PAGE
> 95% as analyzed by HPLC
Endotoxin Level < 0.2 EU/ µg of protein by gel clotting method
Biological Activity ED50 < 10.0 µg/ml, measured by its ability to induce alkaline phosphatase production by CCL-226 cells, corresponding to a specific activity of > 100.0 units/mg.
Expression System E. coli
Formulation Lyophilized after extensive dialysis against PBS.
Reconstitution It is recommended that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Reconstitute the lyophilized powder in ddH₂O up to 100 µg/ml.
Storage & Stability Upon receiving, this product remains stable for up to 6 months at lower than -70°C. Upon reconstitution, the product should be stable for up to 1 week at 4°C or up to 3 months at -20°C. For long term storage it is recommended that a carrier protein (example 0.1% BSA) be added. Avoid repeated freeze-thaw cycles.
Additional Information
Gene ID 50846
Other Names Desert hedgehog protein, DHH, 3.1.-.-, HHG-3, Desert hedgehog protein N-product, DHH-N, DHH (HGNC:2865)
Target Background 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.

For Research Use Only. Not For Use In Diagnostic Procedures.

Protein Information
Name DHH {ECO:0000303|PubMed:33063110, ECO:0000312|HGNC:HGNC:2865}
Function [Desert hedgehog protein]: Precursor of desert hedgehog, a morphogen that activates the smoothened signaling pathway, and which is essential for a variety of patterning events during development (By similarity). The C-terminal part of the precursor displays an autoproteolysis and a cholesterol transferase activity, resulting (1) in the cleavage of the full-length protein into two parts, desert hedgehog protein N-product and C-product and (2) covalent attachment of a cholesterol moiety to the C-terminus of the newly generated N-product (DHH-N) (By similarity). Both autoproteolysis and a cholesterol transferase activities occur in the endoplasmic reticulum (By similarity). Following additional lipidation, DHH-N acts as a morphogen, while the C-product is degraded in the endoplasmic reticulum (By similarity).
Cellular Location [Desert hedgehog protein]: Endoplasmic reticulum membrane {ECO:0000250|UniProtKB:Q15465}. Golgi apparatus membrane {ECO:0000250|UniProtKB:Q15465}. Note=Co-localizes with HHAT in the ER and Golgi membrane. {ECO:0000250|UniProtKB:Q15465}
Research Areas
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