Abstract
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.
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