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The R-spondin protein family

机译:R-spondin蛋白家族

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摘要

The four vertebrate R-spondin proteins are secreted agonists of the canonical Wnt/β-catenin signaling pathway. These proteins are approximately 35 kDa, and are characterized by two amino-terminal furin-like repeats, which are necessary and sufficient for Wnt signal potentiation, and a thrombospondin domain situated more towards the carboxyl terminus that can bind matrix glycosaminoglycans and/or proteoglycans. Although R-spondins are unable to initiate Wnt signaling, they can potently enhance responses to low-dose Wnt proteins. In humans, rare disruptions of the gene encoding R-spondin1 cause a syndrome of XX sex reversal (phenotypic male), palmoplantar keratosis (a thickening of the palms and soles caused by excess keratin formation) and predisposition to squamous cell carcinoma of the skin. Mutations in the gene encoding R-spondin4 cause anonychia (absence or hypoplasia of nails on fingers and toes). Recently, leucine-rich repeat-containing G-protein-coupled receptor (Lgr)4, Lgr5 and Lgr6, three closely related orphans of the leucine-rich repeat family of G-protein-coupled receptors, have been identified as receptors for R-spondins. Lgr5 and Lgr6 are markers for adult stem cells. Because R-spondins are potent stimulators of adult stem cell proliferation in vivo and in vitro, these findings might guide the therapeutic use of R-spondins in regenerative medicine.
机译:四种脊椎动物R-spondin蛋白是经典Wnt /β-catenin信号传导途径的分泌激动剂。这些蛋白质约为35 kDa,其特征在于两个Wnt信号增强所必需和充分的氨基末端弗林蛋白酶样重复序列,以及一个更靠近羧基末端的可与基质糖胺聚糖和/或蛋白聚糖结合的血小板反应蛋白结构域。尽管R-spondins无法启动Wnt信号传导,但它们可以有效增强对低剂量Wnt蛋白的反应。在人类中,罕见的编码R-spondin1的基因破坏会导致XX性逆转综合征(表型男性),掌palm角化病(由于过度的角​​蛋白形成而引起的手掌和足底增厚),以及易患皮肤鳞状细胞癌。编码R-spondin4的基因中的突变会导致甲癣(手指和脚趾上的指甲缺失或发育不全)。最近,已确定富含亮氨酸重复序列的G蛋白偶联受体(Lgr)4,Lgr5和Lgr6是富含亮氨酸重复序列的G蛋白偶联受体的三个密切相关的孤儿。 spondins。 Lgr5和Lgr6是成人干细胞的标志物。由于R-spondins是体内和体外成年干细胞增殖的有效刺激剂,因此这些发现可能指导R-spondins在再生医学中的治疗用途。

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