NOVEL CLASSIFICATION OF RENAL TUBULAR ACIDOSIS BY EXPRESSION OF ACID-BASE RELATED PROTEINS

我们进行了基础研究，试图通过分析酸碱相关蛋白，对肾小管酸中毒进行新的分类。最初，我们研究了肾脏中酸碱相关蛋白的表达。采用抗h - atp酶抗体（Ell）、抗ⅲ带抗体（IVF12）、PNA检测α型插层细胞和β型插层细胞。在新生兔中，肾发生仍然发生，h - atp酶和III带的细胞极性未建立。有趣的是，在Ell和PNA双标记的情况下，Ell和PNA都有，只有Ell，只有PNA标记的细胞在1周分别为（94%,5%,1%），在3周分别为（82%,14%,4%），在成人中分别为（82%,12%,4%）。结果发现，在新生儿中，早产儿细胞没有很好地分化为α型和β型插层细胞。在碳酸酐酶（CA）染色上，所有分化的间充质细胞都含有CA，但随着肾脏成熟，CA逐渐消失。随着年龄的增长，CA可能定位于近端细胞或嵌入细胞。另一方面，临床有几例RTA I患者的h - atp酶染色较正常对照低。同样，一些RTA II患者的h - atp酶染色较弱。在IVF12染色中，本研究研究的患者未显示任何怀疑III带缺乏的免疫细胞化学染色。由于细胞分化不成熟，细胞极性未建立，新生儿易出现酸碱失调。此外，就酸碱相关蛋白而言，h - atp酶和III带的低生成可能部分与RTA有关。在未来，为了更好地理解RTA，需要通过酸负载增加h - atp酶，III带或酸相关蛋白的mRNA。

We have performed the basic research to try to make the novel classification of renal tubular acidosis by analyzing the acid-base related proteins. Initially, we have investigated the expression of acid-base related proteins in the kidney with nephrogenesis. Anti-H-ATPase antibody (Ell), anti-Band III antibody (IVF12), PNA are used for the detection of alphatype intercalated cells and beta type intercalated cells. In the newborn rabbit, nephrogenesis still occurred and cell polality with H-ATPase and Band III were not established. Interestingly, with double labeling of Ell and PNA,both Ell and PNA,only Ell, only PNA labeling cell are (94%, 5%, 1%) in 1 week, (82%, 14%, 4%) in 3weeks, and (82%, 12%, 4%) in adult respectively. It was found that in the newborn premature cells were not well differentiated into alpha and beta type intercalated cells. On carbonic anhydrase (CA) staining, all the differentiating mesenchymal cells contain CA but fade it out with renal maturation. CA supposed to become to localize at proximal cells or intercalated cells with age. On the other hand, clinically several patients with RTA I showed less intense staining of H-ATPase Comparing to normal control. Similarly, some of patients with RTA II showed less intense staining of H-ATpase. In IVF12 staining, the patients studied in this study did not show any lack of immunocytochemical staining which suspect Band III deficiency. Because of the immaturity of cell differentiation and unestblishment of cell polality, newborn infant tended to be acid-base disturbance. Furthermore, with regard to acid-base related proteins, lower production of H-ATPase and Band III may in part relate to RTA.In the future, increment of H-ATPase, Band III,or mRNA of acid-related proteins by acid loading will be required for the better understanding of RTA.