Taniguchi N, Miyawaki T, Moriya N, Okuda N
Clin Chim Acta. 1975 May 1;60(3):363-9. doi: 10.1016/0009-8981(75)90079-0.
Heparan sulfates were isolated from the urine of normal individuals and patients with genetic mucopolysaccharidoses after exhaustive digestion with chondroitinase ABC. Electrophoresis of these preparations on cellulose acetate membrane revealed one spot corresponding in mobility to reference heparan sulphate in barium acetate buffer, while electrophoresis in 0.1 M HCl resulted in two distinct spots for each case; one corresponded in migration rate to reference heparan sulfate, and the other was faster in mobility than reference heparan sulfate but slightly retarded when compared with reference heparin. On thin-layer gel filtration on Sephadex G-200 (superfine) heparan sulfate from normal urine was polydispersed in character and its molecular size was larger than those of other preparations. Heparan sulfates from Hunter's and Sanfilippo's urine were monodispersed and small in molecular size. The molecular size of heparan sulfate from Sanfilippo's urine was the smallest of all. Heparin sulfate from Hurler's urine appeared to be composed of two populations; one corresponded in molecular size to heparan sulfate from normal urine, and the other corresponded to that of Hunter's urine.
用软骨素酶ABC彻底消化后，从正常个体和患有遗传性黏多糖贮积症患者的尿液中分离出硫酸乙酰肝素。这些制剂在醋酸纤维素膜上进行电泳，在醋酸钡缓冲液中显示出一个迁移率与参考硫酸乙酰肝素相对应的斑点，而在0.1 M盐酸中电泳时，每种情况都产生两个明显的斑点；一个迁移率与参考硫酸乙酰肝素相对应，另一个迁移率比参考硫酸乙酰肝素快，但与参考肝素相比迁移略有滞后。在Sephadex G - 200（超细）上进行薄层层析时，正常尿液中的硫酸乙酰肝素具有多分散性，其分子大小比其他制剂的大。来自亨特氏病和桑菲利普氏病患者尿液中的硫酸乙酰肝素是单分散的，分子大小较小。桑菲利普氏病患者尿液中硫酸乙酰肝素的分子大小是所有样本中最小的。胡勒氏病患者尿液中的硫酸乙酰肝素似乎由两部分组成；一部分分子大小与正常尿液中的硫酸乙酰肝素相对应，另一部分与亨特氏病患者尿液中的相对应。
