Induced degradation of glycosaminoglycans in Hurler's and Hunter's syndromes by plasma infusion.
Induced degradation of glycosaminoglycans in Hurler's and Hunter's syndromes by plasma infusion.
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通过血浆输注诱导 Hurler 综合征和 Hunter 综合征中的糖胺聚糖降解。
DOI：
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发表时间：
1971
影响因子：
11.1
通讯作者：
Robert K. Berglund
中科院分区：
文献类型：
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作者：
N. Ferrante;B. Nichols;P. Donnelly;G. Neri;R. Hrgovcic;Robert K. Berglund
对粘多糖病I型和II型（赫勒综合征和亨特综合征）患者给予正常人血浆的效果进行了评估。输注后，尿中相对较大分子量的糖胺聚糖的排泄减少，其降解产物的排泄增加。其中，硫酸皮肤素和硫酸肝素的降解产物得到了证实。结果表明，正常人血浆中可能含有参与硫酸皮肤素和硫酸肝素正常降解的“因子”，而这些因子在患病状态下是缺失的。
The effects of the administration of normal human plasma to patients affected by mucopolysaccharidoses I and II (Hurler's and Hunter's syndromes) have been evaluated. The infusion was followed by a decreased urinary excretion of relatively large molecular weight glycosaminoglycans and by an increased excretion of their products of degradation. Among the latter, products of the degradation of dermatan sulfate and heparan sulfate could be demonstrated. The results indicate that normal human plasma may contain those "factors" that are involved in the normal degradation of dermatan sulfate and heparan sulfate, that are missing in the diseased states.