Ohishi H, Skinner M, Sato-Araki N, Okuyama T, Gejyo F, Kimura A, Cohen A S, Schmid K
Department of Biochemistry, Boston University School of Medicine, MA 02118.
Clin Chem. 1990 Jan;36(1):88-91.
Significant amounts of glycosaminoglycans (GAGs) were found in amyloid fibril preparations. Using two-dimensional electrophoresis to fractionate GAG mixtures, we quantified and identified for the first time the GAGs of the fibrils from carpal synovium of patients with amyloid associated with chronic hemodialysis. The total GAG content was small, but the GAG distribution (high relative content of chondroitin sulfate and hyaluronic acid and lack of the other GAGs) was unique, unlike that for the other amyloid fibril preparations. The amyloid-rich heart, liver, and spleen tissues, as well as the fibrils isolated from these tissues of patients with systemic forms (primary amyloid and secondary amyloid) of amyloid disease, were also analyzed for GAGs. Fibrils from heart tissue of a patient with primary amyloidosis, now examined for the first time, contained four major GAGs (chondroitin sulfate, dermatan sulfate, hyaluronic acid, and heparan sulfate).
在淀粉样原纤维制剂中发现了大量的糖胺聚糖(GAGs)。我们使用二维电泳对GAG混合物进行分离,首次对慢性血液透析相关淀粉样变患者腕关节滑膜的原纤维中的GAGs进行了定量和鉴定。GAG的总含量很少,但GAG的分布(硫酸软骨素和透明质酸的相对含量较高,且缺乏其他GAGs)是独特的,与其他淀粉样原纤维制剂不同。我们还对富含淀粉样蛋白的心脏、肝脏和脾脏组织,以及从患有系统性淀粉样变疾病(原发性淀粉样变和继发性淀粉样变)患者的这些组织中分离出的原纤维进行了GAGs分析。一名原发性淀粉样变患者心脏组织的原纤维,目前首次进行检测,其中含有四种主要的GAGs(硫酸软骨素、硫酸皮肤素、透明质酸和硫酸乙酰肝素)。