Human DHH (C23II) Recombinant Protein (RPPB0908)
- SKU:
- RPPB0908
- Product type:
- Recombinant Protein
- Size:
- 25ug
- Species:
- Human
- Target:
- DHH (C23II)
- Synonyms:
- HHG-3
- Desert Hedgehog homolog
- MGC35145
- Desert hedgehog protein
- Source:
- Escherichia Coli
- Uniprot:
- O43323
Description
| Product Name: | Human DHH (C23II) Recombinant Protein |
| Product Code: | RPPB0908 |
| Size: | 25µg |
| Species: | Human |
| Target: | DHH (C23II) |
| Synonyms: | HHG-3, Desert Hedgehog homolog, MGC35145, Desert hedgehog protein, DHH. |
| Source: | Escherichia Coli |
| Physical Appearance: | Sterile Filtered White lyophilized (freeze-dried) powder. |
| Formulation: | Lyophilized from a 0.2µm filtered concentrated solution in 1×PBS, pH 7.4. |
| Solubility: | It is recommended to reconstitute the lyophilized DHH (C23II) in sterile 18M-cm H2O not less than 100µg/ml, which can then be further diluted to other aqueous solutions. |
| Stability: | Lyophilized DHH (C23II) although stable at room temperature for 3 weeks, should be stored desiccated below -18°C. Upon reconstitution DHH (C23II) should be stored at 4°C between 2-7 days and for future use below -18°C. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).Please prevent freeze-thaw cycles. |
| Purity: | Greater than 95.0% as determined by:(a) Analysis by RP-HPLC.(b) Analysis by SDS-PAGE. |
| Amino Acid Sequence: | IIGPGRGPVG RRRYARKQLV PLLYKQFVPG VPERTLGASG PAEGRVARGS ERFRDLVPNY NPDIIFKDEE NSGADRLMTE RCKERVNALA IAVMNMWPGV RLRVTEGWDE DGHHAQDSLH YEGRALDITT SDRDRNKYGL LARLAVEAGF DWVYYESRNH VHVSVKADNS LAVRAGG |
| Biological Activity: | The Biological Activity was determined by its ability to induce alkaline phosphatase production by C3H/10T1/2 (CCL-226) cells. The expected ED50 for this effect is 15-45 ?g/ml. |
DHH is part of the Hedgehog family which encodes signaling molecules that are involved in regulating morphogenesis. DHH protein is a precursor that is autocatalytically cleaved, the N-terminal portion is soluble and contains the signalling activity while the C-terminal portion is involved in precursor processing. Additionally, 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 DHH protein have been associated with partial gonadal dysgenesis (PGD) accompanied by minifascicular polyneuropathy. DHH plays a role both male gonadal differentiation and perineurial development.DHH plays a role in intercellular signaling which is essential for a variety of patterning events during development. DHH functions as a spermatocyte survival factor in the testes & is essential for testes development.
DHH (C23II) Human Recombinant produced in E.Coli is a single, non-glycosylated polypeptide chain containing 177 amino acids and having a molecular mass of 19.9kDa. The DHH (C23II) is purified by proprietary chromatographic techniques.
| UniProt Protein Function: | DHH: Intercellular signal essential for a variety of patterning events during development. May function as a spermatocyte survival factor in the testes. Essential for testes development. Defects in DHH may be the cause of partial gonadal dysgenesis with minifascicular neuropathy 46,XY (PGD). PGD is characterized by the presence of a testis on one side and a streak or an absent gonad at the other, persistence of Muellerian duct structures, and a variable degree of genital ambiguity. Defects in DHH may be the cause of complete pure gonadal dysgenesis 46,XY type (GDXYM); also known as male- limited gonadal dysgenesis 46,XY. GDXYM is a type of hypogonadism in which no functional gonads are present to induce puberty in an externally female person whose karyotype is then found to be XY. The gonads are found to be non-functional streaks. Belongs to the hedgehog family. |
| UniProt Protein Details: | Protein type:Secreted; Secreted, signal peptide; Cell development/differentiation Chromosomal Location of Human Ortholog: 12q13.1 Cellular Component: extracellular space; plasma membrane Molecular Function:peptidase activity; protein binding; zinc ion binding; patched binding; calcium ion binding Biological Process: myelination; Leydig cell differentiation; cell-cell signaling; male sex determination; regulation of steroid biosynthetic process; proteolysis; spermatid development; response to estradiol stimulus Disease: 46,xy Gonadal Dysgenesis, Partial, With Minifascicular Neuropathy; 46,xy Sex Reversal 7 |
| NCBI Summary: | This gene encodes a member of the hedgehog family. The hedgehog gene family encodes signaling molecules that play an important role in regulating morphogenesis. This protein is predicted to be made as a precursor that is autocatalytically cleaved; the N-terminal portion is soluble and contains the signalling 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. This protein may be involved in both male gonadal differentiation and perineurial development. [provided by RefSeq, May 2010] |
| UniProt Code: | O43323 |
| NCBI GenInfo Identifier: | 6166118 |
| NCBI Gene ID: | 50846 |
| NCBI Accession: | O43323.1 |
| UniProt Secondary Accession: | O43323,Q15794, |
| UniProt Related Accession: | O43323 |
| Molecular Weight: | 43,577 Da |
| NCBI Full Name: | Desert hedgehog protein |
| NCBI Synonym Full Names: | desert hedgehog |
| NCBI Official Symbol: | DHH |
| NCBI Official Synonym Symbols: | GDXYM; HHG-3; SRXY7 |
| NCBI Protein Information: | desert hedgehog protein; mutant desert hedgehog; desert hedgehog homolog |
| UniProt Protein Name: | Desert hedgehog protein |
| UniProt Synonym Protein Names: | HHG-3 |
| Protein Family: | Desert hedgehog protein |
| UniProt Gene Name: | DHH |
| UniProt Entry Name: | DHH_HUMAN |
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