Ozaki K, Maeda H, Uechi S, Yamate H, Nishikawa T, Nishimura M, Narama I
Research Institute of Drug Safety, Setsunan University, Osaka, Japan.
Exp Mol Pathol. 1994 Dec;61(3):221-9. doi: 10.1006/exmp.1994.1039.
Chediak-Higashi syndrome (CHS) is an autosomal recessive disorder, characterized histopathologically by giant granules in various cells. Since little is known about the juxtaglomerular (JG) cells of CHS, the present study was undertaken as a morphological investigation of the JG cell of Beige rats, which were found to be a new animal model of CHS. In addition, we measured the blood pressure and plasma renin activity. The blood pressure of Beige rats is similar to that of DA rats, the mother strain of Beige rats. However, the plasma renin activity of Beige rats increases with age, whereas that of DA rats decreases. Morphologically, JG cells of Beige rats gave no evidence of hyperplasia and hypertrophy. The most conspicuous change was a small number of giant JG granules. Ultrastructurally, JG granules of Beige rats were large, irregular, and varied in size at 2 months of age, but no age-dependent changes were detected. These results suggested that giant JG granule formation slightly increases renin release with age, but does not affect blood pressure.
切迪阿克-希加什综合征（CHS）是一种常染色体隐性疾病，其组织病理学特征为各种细胞中出现巨大颗粒。由于对CHS的球旁（JG）细胞了解甚少，本研究以米色大鼠的JG细胞为对象进行形态学研究，米色大鼠被发现是CHS的一种新动物模型。此外，我们测量了血压和血浆肾素活性。米色大鼠的血压与米色大鼠的母系品系DA大鼠相似。然而，米色大鼠的血浆肾素活性随年龄增长而增加，而DA大鼠的血浆肾素活性则下降。形态学上，米色大鼠的JG细胞没有增生和肥大的迹象。最明显的变化是少量巨大的JG颗粒。超微结构上，米色大鼠的JG颗粒在2月龄时体积大、不规则且大小不一，但未检测到与年龄相关的变化。这些结果表明，巨大JG颗粒的形成随年龄增长会轻微增加肾素释放，但不影响血压。
