Devuyst O
Bull Mem Acad R Med Belg. 1999;154(6 Pt 2):309-18.
The molecular identification of several transporters has allowed a better understanding of the mechanism involved in cyst fluid accumulation in the autosomal dominant polycystic kidney disease (ADPKD). This fluid secretion is mediated by Cl- secretion, the latter process being stimulated by cyclic AMP. At the cellular level, Cl- secretion is carried out by a basolateral, bumetanide-sensitive cotransporter, organized in series with an apical CFTR channel. The accumulation of Cl- in the cyst lumen attracts Na+ (electric coupling) and water (osmotic coupling), the movement of the latter being partially mediated by aquaporins. The understanding of cyst fluid secretion mechanisms will be helpful to elaborate new therapeutic strategies aiming at slowing cyst expansion and preserving renal function.
几种转运蛋白的分子鉴定有助于更好地理解常染色体显性多囊肾病（ADPKD）中囊肿液积聚所涉及的机制。这种液体分泌由氯离子分泌介导，后者过程由环磷酸腺苷刺激。在细胞水平上，氯离子分泌由基底外侧布美他尼敏感的协同转运蛋白完成，该蛋白与顶端囊性纤维化跨膜传导调节因子（CFTR）通道串联排列。氯离子在囊肿腔内的积聚吸引钠离子（电偶联）和水（渗透偶联），水的移动部分由水通道蛋白介导。对囊肿液分泌机制的理解将有助于制定旨在减缓囊肿扩张和保护肾功能的新治疗策略。
