RSPO1 is a member of the R-spondin family and encodes a secreted activator protein with two cystein-rich, furin-like domains and one thrombospondin type 1 domain. In mice, the protein induces the rapid onset of crypt cell proliferation and increases intestinal epithelial healing, providing a protective effect against chemotherapy-induced adverse effects.
产品应用
应用
已检合格种属
预测种属
推荐稀释比例
IHC-P
Rat
Human, Rabbit, Pig, Dog
1:100-500
IHC-F
Rat
Human, Rabbit, Pig, Dog
1:100-500
IF
Rat
Human, Rabbit, Pig, Dog
1:100-500
交叉反应
交叉反应: Rat (predicted: Human, Rabbit, Pig, Dog)
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靶标
基因名
RSPO1
蛋白名
R-spondin-1
亚基
Interacts with the extracellular domain of FZD8 and LRP6. It however does not form a ternary complex with FZD8 and LRP6. Interacts with WNT1. Binds heparin (By similarity).
亚细胞定位
Secreted.
组织特异性
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.
疾病
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.
相似性
Belongs to the R-spondin family. Contains 2 FU (furin-like) repeats. Contains 1 TSP type-1 domain.
功能
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.