Pflumio F, Fonteneau P, Loor F
Laboratoire d'Immunologie, Université Louis Pasteur Strasbourg I, Illkirch, France.
Immunol Lett. 1990 Feb;23(4):269-74. doi: 10.1016/0165-2478(90)90071-w.
The murine equivalent of the human Chediak-Higashi Syndrome is the beige (bg) mutant in the C57BL/6 (B6) background. Besides the well-known lack of natural killer (NK) activity in bg-homozygous mice, functional abnormalities of T cells, macrophages and various granulocytes have been reported. With the exception of one study indicating a decreased in vitro response to lipopolysaccharide, there is no report concerning the B cell compartment of the beige mutant. The in vivo anti-trinitrophenyl antibody response to a TI-2 antigen (TNP-Ficoll) was found here to be significantly lower in B6 beige than in B6 wild mice, although both strains responded similarly to an analogous TD antigen (TNP-ovalbumin). Since the marginal zone macrophages of the spleen were previously shown to be essential for the initiation of antibody responses to TI-2 antigens, they might be another target of the beige mutation.
人类切-东综合征（Chediak-Higashi Syndrome）在小鼠中的对应类型是C57BL/6（B6）背景下的米色（bg）突变体。除了众所周知的bg纯合小鼠缺乏自然杀伤（NK）活性外，还报道了T细胞、巨噬细胞和各种粒细胞的功能异常。除了一项研究表明对脂多糖的体外反应降低外，没有关于米色突变体B细胞区室的报道。在这里发现，B6米色小鼠对TI-2抗原（TNP-菲可）的体内抗三硝基苯抗体反应明显低于B6野生小鼠，尽管这两个品系对类似的TD抗原（TNP-卵清蛋白）的反应相似。由于先前已证明脾脏边缘区巨噬细胞对于启动针对TI-2抗原的抗体反应至关重要，它们可能是米色突变的另一个靶点。
