General Description
R-Spondin-1 (Rspo-1) belongs to the (Rspo) family of Wnt modulators. Currently, the (Rspo) family consists of four structurally related secreted ligands (Rspo 1-4), all containing furin-like and thrombospondin structural domains. Rspo-1 is expressed in certain areas of the developing central nervous system, as well as in adrenal glands, ovary, testis, thyroid, and trachea. Recombinant human R-Spondin-1 is a 26.7kDa protein consisting of 243 amino acid residues. Due to glycosylation, R-Spondin-1 migrates at an apparent molecular weight of approximately 40.0kDa by SDS PAGE analysis under reducing conditions.
Biochem/physiol Actions
Rspo (R-Spondin) can interact with the Frizzled/LRP6 (LDL receptor related protein 6) receptor complex in a manner that stimulates the Wnt/beta-catenin signaling pathway. Disruption of Rspo-1 gene causes recessive syndrome with XX sex reversal, palmoplantar hyperkeratosis and predisposition to squamous cell carcinoma of the skin. Rspo-1 is also associated with radioresistance of tumor cells.
