Ozaki K, Maeda H, Nishikawa T, Nishimura M, Narama I
Research Institute of Drug Safety, Setsunan University, Osaka, Japan.
J Comp Pathol. 1994 May;110(4):369-79. doi: 10.1016/s0021-9975(08)80314-3.
Chediak-Higashi syndrome (CHS) is a rare disease occurring in several animal species. Recently, mutant beige rats with CHS were found among DA strain rats in Japan. In the present study, histological examination of beige rats revealed giant granules in the hepatocytes, renal proximal tubules, submandibular ducts, thyroid follicular cells, granulocytes, mast cells, melanocytes, retinal pigment epithelial cells and globular leucocytes. Ultrastructurally, these granules varied from enlarged lysosomes, which were amorphous, granular or filamentous, to giant mast cell granules, crystalloid granules of eosinophils and slightly enlarged neutrophil granules. These findings bore many similarities to those in the beige mouse, which is a well known animal model for CHS, but some differences were apparent. Thus the giant granules of beige rats were larger and more easy to observe than those in beige mice. The study indicated that the beige rat may prove useful as an animal model for CHS.
切迪阿克-希加希综合征（CHS）是一种在多种动物物种中出现的罕见疾病。最近，在日本的DA品系大鼠中发现了患有CHS的突变米色大鼠。在本研究中，对米色大鼠的组织学检查显示，肝细胞、肾近端小管、下颌下导管、甲状腺滤泡细胞、粒细胞、肥大细胞、黑素细胞、视网膜色素上皮细胞和球形白细胞中存在巨大颗粒。在超微结构上，这些颗粒从扩大的溶酶体（无定形、颗粒状或丝状）到巨大的肥大细胞颗粒、嗜酸性粒细胞的晶体颗粒和略微扩大的中性粒细胞颗粒不等。这些发现与作为CHS著名动物模型的米色小鼠有许多相似之处，但也有一些明显的差异。因此，米色大鼠的巨大颗粒比米色小鼠的更大且更易于观察。该研究表明，米色大鼠可能被证明是一种有用的CHS动物模型。
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