Dean M F, Muir H
Biochem J. 1970 Aug;118(5):783-90. doi: 10.1042/bj1180783.
Kurloff cells of guinea pigs increase in number and accumulate in the spleen on oestrogen treatment. Because they contain metachromatic inclusions and are considered to be lymphocytes they were examined as a possible model for mucopolysaccharidoses like Hurler's syndrome, where some lymphocytes are also metachromatic. Oestrogen treatment produced a large increase in a glycosaminoglycan resembling chondroitin 4-sulphate in chemical analysis, chromatographic behaviour and i.r. spectrum but with an additional strong band at 805cm(-1). Material isolated without proteolysis behaved on gel chromatography as a multiple-chain protein-polysaccharide whose molecular size was decreased by proteolysis. It contained xylose and galactose in molar proportions with serine, compatible with the presence of the same linkage region as in cartilage chondroitin 4-sulphate proteins and which likewise underwent alkaline beta-elimination. Kurloff glycosaminoglycan chains were significantly longer than chondroitin sulphate chains of cartilage protein-polysaccharides as assessed by gel chromatography and the molar ratios of galactosamine to xylose or to serine. Kurloff cells thus contain intact rather than partially degraded protein-polysaccharide and hence are not analogous to Hurler cells, and their electron micrographs were also different. The purified Kurloff protein-polysaccharide and glycosaminoglycan isolated here has been shown by Marshall, Swettenham, Vernon-Roberts & Revell (1970) to be toxic specifically to macrophages at extremely low concentrations in vitro, unlike chondroitin sulphate of protein-polysaccharides from cartilage. The toxic constituent may account for the i.r.-absorption band at 805cm(-1). Although active incorporation of [(35)S]sulphate occurs at early stages of Kurloff-cell induction (Marshall et al. 1970), the fully developed Kurloff cell studied here showed very low incorporation in vitro and in vivo, suggesting that the inclusions are specialized for the storage of the toxic material.
豚鼠的库尔洛夫细胞在雌激素处理后数量增加并积聚于脾脏。由于它们含有异染性包涵体且被认为是淋巴细胞，所以被作为黏多糖贮积症（如胡尔勒综合征）的一种可能模型进行研究，在胡尔勒综合征中一些淋巴细胞也是异染性的。雌激素处理使一种在化学分析、色谱行为和红外光谱方面类似于硫酸软骨素4的糖胺聚糖大量增加，但在805cm⁻¹处有一条额外的强带。未经蛋白水解分离得到的物质在凝胶色谱上表现为一种多链蛋白多糖，其分子大小经蛋白水解后减小。它含有木糖和半乳糖，其摩尔比例与丝氨酸相符，这与软骨硫酸软骨素4蛋白中存在的相同连接区域一致，并且同样会发生碱性β消除反应。通过凝胶色谱以及半乳糖胺与木糖或丝氨酸的摩尔比评估，库尔洛夫糖胺聚糖链明显长于软骨蛋白多糖的硫酸软骨素链。因此，库尔洛夫细胞含有完整的而非部分降解的蛋白多糖，所以与胡尔勒细胞不同，它们的电子显微照片也不一样。马歇尔、斯韦滕纳姆、弗农 - 罗伯茨和雷维尔（1970年）已表明，这里分离得到的纯化库尔洛夫蛋白多糖和糖胺聚糖在体外极低浓度下对巨噬细胞具有特异性毒性，这与软骨蛋白多糖的硫酸软骨素不同。这种有毒成分可能是805cm⁻¹处红外吸收带的原因。尽管在库尔洛夫细胞诱导的早期阶段会发生[(³⁵)S]硫酸盐的活跃掺入（马歇尔等人，1970年），但这里研究的完全发育的库尔洛夫细胞在体外和体内的掺入率都非常低，这表明这些包涵体专门用于储存有毒物质。
