肾素家族相互作用
中文名称
通路描述
肾素（NPHS1）是超 IgG 分子家族的一员，主要在肾小管足细胞中表达。它是 slit 小体（一种细胞间修饰的粘附连接）的主要甚至最重要的结构成分。NPHS1 具有一个包含八个远端 IgG 样结构和一个近端纤连蛋白 III 结构域的胞外结构域，一个跨膜结构域和一个短的胞内结构域。NPHS1 分子表现出同性和异性的相互作用。在异质性相互作用伙伴中， slit 小体蛋白，如 KIRREL（IRRE 样蛋白 1）、NEPH1（肾素样蛋白 1）和 KIRREL3（NEPH2）或 KIRREL2（NEPH3），被证明稳定 slit 小体结构。胞内 Podocin（NPHS2）、CD2 关联蛋白（CD2AP）和粘附连接蛋白如 IQGAP、MAGI、CASK 和 spectrins 都与 NPHS1 相互作用。因此，它似乎对组织 slit 小体本身的分子结构起主要作用，并通过其与结合伙伴的结合将其与肌动蛋白细胞骨架联系起来。Src 激酶 FYN 对 NPHS1 的酪氨酸磷酸化启动 PI3K-AKT 信号级联，似乎促进抗凋亡信号。
英文描述
Nephrin family interactions Nephrin (NPHS1) is a member of the Super-IgG-Molecule family and is most prominently expressed in kidney podocytes. It is a major if not the most important structural component of the slit diaphragm, a modified adherens junction in between these cells. NPHS1 has an extracellular domain that contains eight distal IgG like domains and one proximal fibronectin type III domain, a transmembrane domain and a short intracellular domain. NPHS1 molecules show both homophilic and heterophilic interactions. Among heterophilic interaction partners, slit diaphragm proteins such as Kin of IRRE-like protein 1 (KIRREL, Nephrin-like protein 1, NEPH1), KIRREL3 (NEPH2) and KIRREL2 (NEPH3) were shown to stabilize the slit diaphragm structure. Intracellularly Podocin (NPHS2), CD2 associated protein (CD2AP) and adherins junction associated proteins like IQGAP, MAGI, CASK and spectrins all interact with NPHS1. Hence it seems to play a major role in organizing the molecular structure of the slit diaphragm itself and via its binding partners links it to the actin cytoskeleton. NPHS1 tyrosine phosphorylation by the Src kinase FYN initiates the PI3K-AKT signaling cascade, which seems to promote antiapoptotic signals.
所含基因
23 个基因