Human R-spondin-1(RSPO1) ELISA kit

Activator of the canonical Wnt signaling pathway by acting as a ligand for LGR4-6 receptors. Upon binding to LGR4-6 (LGR4, LGR5 or LGR6), LGR4-6 associate with phosphorylated LRP6 and frizzled receptors that are activated by extracellular Wnt receptors, triggering the canonical Wnt signaling pathway to increase expression of target genes. Also regulates the canonical Wnt/beta-catenin-dependent pathway and non-canonical Wnt signaling by acting as an inhibitor of ZNRF3, an important regulator of the Wnt signaling pathway. Acts as a ligand for frizzled FZD8 and LRP6. May negatively regulate the TGF-beta pathway. Has a essential roles in ovary determination. Regulates Wnt signaling by antagonizing DKK1/KREM1-mediated internalization of LRP6 through an interaction with KREM1.

1. first report of a RSPO1 missense mutation in association with human disease (familial 46,XX disorder of sexual development) . PMID: 29262419
2. Rspo1 increases the number of Lgr5(+) liver stem cells in human liver fibrosis tissues, and once they are isolated, these cells are able to form organoids, and treatment with HGF/Rspo1 promotes their expansion PMID: 29079780
3. RSPOs facilitate HSC activation and promote liver fibrogenesis by enhancing the Wnt pathway PMID: 27572318
4. Role of RSPO1 in gastric cancer PMID: 28219935
5. Results showed that Rspo1 expression is downregulated in adult follicles but its activation is sufficient in promoting ovarian tumors supporting its direct involvement in the initiation of ovarian cancers. PMID: 27270435
6. We highlight the cooperation of WNT4, RSPO1 and FOXL2 within a regulatory network and the need for further research to better understand their role in defining and maintaining ovarian identity. PMID: 27604691
7. Rspo1 glycosylation at Asn137 is essential for secretion and stability but not for heparin binding. PMID: 27314333
8. DPY19L3-mediated C-mannosylation of Rspo1 at tryptophan(156) is required for Rspo1 secretion. PMID: 26764097
9. Genetic variants in ZC3H11B, RSPO1, and GJD2 are associated with susceptibility to the development of high myopia in a Han Chinese population. PMID: 26485405
10. R-Spondin1 has an effect on radiosensitivity of glioma cells PMID: 25865226
11. ZNRF3 and LGR4-binding sites in RSPO1 are required for Wnt signaling. PMID: 24165923
12. Changes in the expression levels of IRS1, IRS2, RIPK2, RSPO1, and DNA JC15 genes might contribute to the development of insulin resistance and glucose intolerance in the obese boys. PMID: 26040030
13. the LGR4-Rspo1 complex crystal structure shows divergent mechanisms of ligand recognition by leucine-rich repeat G-protein-coupled receptors PMID: 25480784
14. ZNRF3 binds RSPO1 and LGR5-RSPO1 with micromolar affinity via RSPO1 furin-like 1 (Fu1) domain. PMID: 24349440
15. overexpressed in fibrotic liver tissue PMID: 25218283
16. Loss of RSPO1 expression is associated with invasive ductal carcinoma of the breast. PMID: 24373193
17. RSPO-LGR4 not only induces the clearance of RNF43/ZNRF3 to increase Wnt receptor levels but also recruits IQGAP1 into the Wnt signaling complex. PMID: 24639526
18. Crystal structures of the Lgr4 ectodomain alone and bound to Rspo1. PMID: 23891289
19. In conclusion, present study highlights the role of Rspo 1 in bone remodeling where it activates Wnt signaling to induce differentiation, as shown in human as well murine in vitro osteoblast cell models. PMID: 23617070
20. Recent developments have demonstrated that ovarian development is an active process (rather than a default process); ovarian development/function requires expression of RSPO1, WNT4, and FOXL2. [REVIEW] PMID: 23044875
21. R-Spondin potentiates Wnt/beta-catenin signaling through orphan receptors LGR4 and LGR5 PMID: 22815884
22. study provides new mechanistic insights into the regulation of Wnt receptor turnover, and reveals ZNRF3 as a tractable target for therapeutic exploration PMID: 22575959
23. R-spondin1 is upregulated during critical stages of early human ovary development and may function as a tissue-specific amplifier of beta-catenin signaling to oppose testis determination. PMID: 21297984
24. identified a gene, R-spondin1, with potent and specific proliferative effects on intestinal crypt cells PMID: 16109882
25. Human R-spondin1 (RSPO1) is the gene disrupted in a recessive syndrome characterized by XX sex reversal, palmoplantar hyperkeratosis and predisposition to squamous cell carcinoma of the skin. PMID: 17041600
26. RSPO1 regulates Wnt signaling by inhibiting internalization of LRP6. PMID: 17804805
27. Mutational analysis of the RSPO1 gene in a 46,XX woman with true hermaphroditism, palmoplantar keratoderma, congenital bilateral corneal opacities, onychodystrophy, and hearing impairment, is reported. PMID: 18085567
28. R-sponin1 (Rspo1) acted synergistically with Wnt3A to activate Wnt/beta-catenin signaling in the uncommitted mesenchymal C2C12 cells. PMID: 18242177
29. SRY represses the transcriptional of the Rspo1/Wnt target genes involved in ovarian determination. PMID: 19376480
30. Mice treated with R-spondin1 showed increased intestinal epithelial healing, providing a protective effect against chemotherapy-induced intestinal mucositis. PMID: 16306530

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Keratoderma, palmoplantar, with squamous cell carcinoma of skin and sex reversal (PKKSCC)

Secreted. Nucleus.

R-spondin family

Abundantly expressed in adrenal glands, ovary, testis, thyroid and trachea but not in bone marrow, spinal cord, stomach, leukocytes colon, small intestine, prostate, thymus and spleen.

HGNC: 21679

OMIM: 609595

KEGG: hsa:284654

STRING: 9606.ENSP00000348944

UniGene: Hs.135015