Stenstad T, Magnus J H, Kolset S O, Cornwell G G, Husby G
Department of Rheumatology, University of Tromsø, Norway.
Scand J Immunol. 1991 Nov;34(5):611-7. doi: 10.1111/j.1365-3083.1991.tb01584.x.
We have previously demonstrated the presence of glycosaminoglycans (GAGs) in water extracts of secondary AA amyloid fibrils. In the present study we isolated significant quantities of GAGs from fibril extracts of immunoglobulin light chain (AL) type derived from the spleens from two patients afflicted with primary amyloidosis. Employing ion-exchange chromatography and gel filtration subsequent to various specific chemical and enzymatic treatments, different types of high molecular weight GAGs were found in both preparations, but not in the corresponding normal splenic extracts. The amyloid-associated GAGs of the extracts derived from one patient consisted of 60% dermatan sulphate and 40% heparan sulphate whereas those obtained from the second spleen were 25% dermatan sulphate and 75% heparan sulphate. The heparan sulphate fraction occurred in the form of proteoglycans, whereas the dermatan sulphate apparently occurred as free GAG chains, resembling the data recently obtained from AA amyloid fibril extracts.
我们之前已证实在继发性AA淀粉样原纤维的水提取物中存在糖胺聚糖（GAGs）。在本研究中，我们从两名原发性淀粉样变性患者脾脏来源的免疫球蛋白轻链（AL）型原纤维提取物中分离出了大量GAGs。在经过各种特定化学和酶处理后，采用离子交换色谱法和凝胶过滤法，在两种提取物中均发现了不同类型的高分子量GAGs，但在相应的正常脾脏提取物中未发现。来自一名患者提取物的淀粉样相关GAGs由60%的硫酸皮肤素和40%的硫酸乙酰肝素组成，而从第二个脾脏获得的GAGs则为25%的硫酸皮肤素和75%的硫酸乙酰肝素。硫酸乙酰肝素部分以蛋白聚糖的形式存在，而硫酸皮肤素显然以游离GAG链的形式存在，这与最近从AA淀粉样原纤维提取物中获得的数据相似。
