O'Neil D C, Bartholomew W R, Rattazzi M C
Biochim Biophys Acta. 1979 Sep 29;580(1):1-9. doi: 10.1016/0005-2795(79)90191-0.
The immunological characteristics of feline beta-hexosaminidase (beta-D-N-acetylglucosaminidase, EC 3.2.1.30) isoenzymes, Hex A and Hex B, were studied. Immunization of rabbits and goats with either cat Hex A or Hex B produced antibodies which reacted with a common antigenic marker shared by both Hex A and Hex B. With properly absorbed antisera, a unique antigenic marker was demonstrated on cat Hex A, but not on Hex B. This antigenic profile is comparable to that of the human beta-hexosaminidase isozymes, in which both Hex A and Hex B share the antigenic determinant, beta, while only Hex A possesses the antigenic determinant, alpha. Ho cross-reactivity between the two species could be demonstrated using goat or rabbit antisera to either feline of human beta-hexosaminidase. These immunological data validate feline Gm2 gangliosidosis as a model for human Gm2 gangliosidosis type II, and facilitate the investigation of enzyme replacement therapy.
对猫β-己糖胺酶(β-D-N-乙酰氨基葡萄糖苷酶,EC 3.2.1.30)同工酶Hex A和Hex B的免疫学特性进行了研究。用猫Hex A或Hex B免疫兔子和山羊可产生与Hex A和Hex B共有的一种共同抗原标志物发生反应的抗体。使用适当吸收的抗血清,在猫Hex A上显示出一种独特的抗原标志物,而在Hex B上未显示。这种抗原谱与人类β-己糖胺酶同工酶的抗原谱相当,其中Hex A和Hex B都具有抗原决定簇β,而只有Hex A具有抗原决定簇α。使用针对猫或人β-己糖胺酶的山羊或兔子抗血清,未发现这两个物种之间有交叉反应。这些免疫学数据证实猫GM2神经节苷脂病可作为人类II型GM2神经节苷脂病的模型,并有助于酶替代疗法的研究。