功能: disease:Defects in RSPO1 are the cause of palmoplantar keratoderma with squamous cell carcinoma of skin and sex reversal (PKKSCC) [MIM:610644]. This recessive syndrome is characterized by XX (female to male) SRY-independent sex reversal, palmoplantar hyperkeratosis and predisposition to squamous cell carcinoma of the skin.,domain:The FU repeats are required for activation and stabilization of beta-catenin.,function:Activator of the beta-catenin signaling cascade, leading to TCF-dependent gene activation. Acts both in the canonical Wnt/beta-catenin-dependent pathway, possibly via a direct interaction with Wnt proteins, and in a Wnt-independent beta catenin pathway through a receptor signaling pathway that may not use frizzled/LRP receptors. Acts as a ligand for frizzled FZD8 and LRP6. May negatively regulate the TGF-beta pathway. Has a essential roles in ovary determination.,miscellaneous:Upon injection into mice, it induces rapid onset of crypt cell proliferation involving beta-catenin stabilization. It also displays efficacy in a model of chemotherapy-induced intestinal mucositis suggesting possible therapeutic application in gastrointestinal diseases.,similarity:Belongs to the R-spondin family.,similarity:Contains 1 TSP type-1 domain.,similarity:Contains 2 FU (furin-like) repeats.,subunit:Interacts with the extracelular domain of FZD8 and LRP6. It however does not form a ternary complex with FZD8 and LRP6. Interacts with WNT1. Binds heparin.,tissue specificity:Abundantly expressed in adrenal glands, ovary, testis, thyroid and trachea but not in bonre marrow, spinal cord, stomach, leukocytes colon, small intestine, prostate, thymus and spleen.,
相关产品: RS0001,RS0002,YM3028,YM3029
细胞定位: Secreted . Nucleus . Seems to mainly localize to nucleoli. .
组织表达: 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.
功能: disease:Defects in RSPO1 are the cause of palmoplantar keratoderma with squamous cell carcinoma of skin and sex reversal (PKKSCC) [MIM:610644]. This recessive syndrome is characterized by XX (female to male) SRY-independent sex reversal, palmoplantar hyperkeratosis and predisposition to squamous cell carcinoma of the skin.,domain:The FU repeats are required for activation and stabilization of beta-catenin.,function:Activator of the beta-catenin signaling cascade, leading to TCF-dependent gene activation. Acts both in the canonical Wnt/beta-catenin-dependent pathway, possibly via a direct interaction with Wnt proteins, and in a Wnt-independent beta catenin pathway through a receptor signaling pathway that may not use frizzled/LRP receptors. Acts as a ligand for frizzled FZD8 and LRP6. May negatively regulate the TGF-beta pathway. Has a essential roles in ovary determination.,miscellaneous:Upon injection into mice, it induces rapid onset of crypt cell proliferation involving beta-catenin stabilization. It also displays efficacy in a model of chemotherapy-induced intestinal mucositis suggesting possible therapeutic application in gastrointestinal diseases.,similarity:Belongs to the R-spondin family.,similarity:Contains 1 TSP type-1 domain.,similarity:Contains 2 FU (furin-like) repeats.,subunit:Interacts with the extracelular domain of FZD8 and LRP6. It however does not form a ternary complex with FZD8 and LRP6. Interacts with WNT1. Binds heparin.,tissue specificity:Abundantly expressed in adrenal glands, ovary, testis, thyroid and trachea but not in bonre marrow, spinal cord, stomach, leukocytes colon, small intestine, prostate, thymus and spleen.,
相关产品: RS0001,RS0002,YM3028,YM3029
细胞定位: Secreted . Nucleus . Seems to mainly localize to nucleoli. .
组织表达: 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.