Strausbauch P, Sawyer R, Volkman A
J Reticuloendothel Soc. 1982 Apr;31(4):361-5.
One phenotypic expression of the Chediak-Higashi syndrome in humans and the analogous disorder in the beige mouse (C57BL/6bg/bg) is the presence of anomalous lysosomes identifiable as enlarged cytoplasmic inclusions. This alteration has been used in the study of the circulation and source of certain mononuclear phagocytes in mice. In the present study abnormally enlarged lysosomes are inconsistently demonstrable in the resident peritoneal macrophage of the beige mouse. However, the beige phenotype is also expressed in resident peritoneal macrophages by the presence of anomalous ring form and elongated dumbbell-shaped lysosomes. The former structures resemble those recently described [13] in blood monocytes from patients with the Chediak-Higashi syndrome.
人类的切迪雅克-东综合征(Chediak-Higashi syndrome)以及米色小鼠(C57BL/6bg/bg)中的类似病症的一种表型表现是存在可识别为扩大的细胞质内含物的异常溶酶体。这种改变已被用于研究小鼠中某些单核吞噬细胞的循环和来源。在本研究中,在米色小鼠的常驻腹膜巨噬细胞中,异常扩大的溶酶体并不能始终如一地被检测到。然而,米色表型在常驻腹膜巨噬细胞中也表现为存在异常的环形和细长哑铃形溶酶体。前者的结构类似于最近在切迪雅克-东综合征患者的血液单核细胞中所描述的[13]。