Nahm M H, Blaese R M, Crain M J, Briles D E
J Immunol. 1986 Dec 1;137(11):3484-7.
The Wiskott-Aldrich Syndrome (WAS) in humans has a number of similarities to the immunodeficiencies found in CBA/N mice, including X-chromosome-linked inheritance, inability to produce antibodies to various carbohydrate antigens, susceptibility to various bacterial infections, and an imbalance in B lymphocyte subpopulations. Moreover, in both man and mice, IgG antibodies to polysaccharides are predominantly, but not exclusively, restricted to a single IgG subclass--IgG2 in man, and IgG3 in the mouse. Because CBA/N mice have a deficiency of IgG3 antibodies and because human IgG2 subclass deficiencies have been generally associated with inability to produce antibodies to carbohydrate antigens, it would seem likely that patients with WAS would have greatly reduced levels of IgG2. Quite to the contrary, the data presented here demonstrate that WAS patients have normal levels of the different IgG subclasses, including IgG2. Thus, inability to produce antibodies to carbohydrates is not always associated with IgG2 subclass deficiency.
人类的威斯科特-奥尔德里奇综合征(WAS)与CBA/N小鼠的免疫缺陷有许多相似之处,包括X染色体连锁遗传、无法产生针对各种碳水化合物抗原的抗体、易受各种细菌感染以及B淋巴细胞亚群失衡。此外,在人和小鼠中,针对多糖的IgG抗体主要但并非唯一地局限于单个IgG亚类——人类为IgG2,小鼠为IgG3。由于CBA/N小鼠缺乏IgG3抗体,且人类IgG2亚类缺陷通常与无法产生针对碳水化合物抗原的抗体相关,因此WAS患者的IgG2水平似乎可能会大幅降低。恰恰相反,此处呈现的数据表明WAS患者不同IgG亚类的水平正常,包括IgG2。因此,无法产生针对碳水化合物的抗体并不总是与IgG2亚类缺陷相关。