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从一名范科尼综合征患者分离出的轻链蛋白诱导上皮紧密连接

Induction of epithelial tight junctions by a light chain protein isolated from a patient with Fanconi's syndrome.

作者信息

Alavi N, Lianos E A, Palant C E, Bentzel C J

出版信息

Nephron. 1983;35(2):130-5. doi: 10.1159/000183061.

Abstract

Tight junctions have not been described in the adult human glomerulus. In a 22-year-old patient with kappa light chain proteinuria, tight junctions were observed between glomerular epithelial cell foot processes (kidney biopsy). The light chain isolated from urine proved to have a relatively high isoelectric point. When the light chain was exposed in vitro to the mucosal surface of a 'leaky' epithelium, the Necturus gallbladder, transepithelial resistance and potential difference increased in a concentration-dependent, reversible manner and NaCl dilution potentials decreased, consistent with a reduction in tight-junctional ionic permeability. Gallbladders fixed in situ and freeze-fractured during peak electrophysiological responses revealed an increase in tight-junctional depth. This report indicates that certain light chains, possibly by virtue of a positive charge, may induce changes in epithelial tight junction structure and/or permeability.

摘要

紧密连接在成人人类肾小球中尚未见报道。在一名22岁的κ轻链蛋白尿患者中,在肾小球上皮细胞足突之间观察到了紧密连接(肾脏活检)。从尿液中分离出的轻链被证明具有相对较高的等电点。当该轻链在体外暴露于“渗漏性”上皮(美西螈胆囊)的黏膜表面时,跨上皮电阻和电位差以浓度依赖性、可逆的方式增加,而NaCl稀释电位降低,这与紧密连接离子通透性的降低一致。在电生理反应峰值期间原位固定并冷冻断裂的胆囊显示紧密连接深度增加。本报告表明,某些轻链可能凭借正电荷诱导上皮紧密连接结构和/或通透性的变化。

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