ACID-HYDROLASES IN SERUM FROM PATIENTS WITH LYSOSOMAL DISORDERS
ACID-HYDROLASES IN SERUM FROM PATIENTS WITH LYSOSOMAL DISORDERS
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DOI：
10.1016/0009-8981(80)90182-5
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发表时间：
1980-01-01
影响因子：
5
通讯作者：
OCKERMAN, PA
中科院分区：
文献类型：
--
作者：
HULTBERG, B;ISAKSSON, A;OCKERMAN, PA
研究了粘脂沉积症（II型和III型）和其他溶酶体疾病患者血清中酸性水解酶的活性。在粘脂沉积症II和III中，除了α-淀粉酶以外，葡糖苷酶，β-葡萄糖苷酶和酸性磷酸酶的活性显著增加。β的高值-半乳糖苷酶见于粘多糖样沉积症I型和II型。戈谢病、青少年黑蒙性白痴和异染性脑白质营养不良。N-乙酰基-β-粘多糖沉积症I、II、III型和戈谢病中氨基葡萄糖苷酶活性高。β的活性-葡萄糖醛酸酶在I、II和III型粘多糖沉积症、戈谢病、青少年黑蒙性白痴和异染性白质营养不良中升高。酸性磷酸酶仅在戈谢病中活性增加。在几种溶酶体贮积症中未发现升高的值。溶酶体贮积症患者（不包括粘脂沉积症II和III）血清中的高值可能取决于肝细胞损伤，这干扰了血清中酸性水解酶的清除。
The activity of acid hydrolases was studied in serum from patients with mucolipidosis (II and III) and other lysosomal disorders. In mucolipidosis II and III all hydrolases examined except .alpha.-glucosidase, .beta.-glucosidase and acid phosphatase were greatly increased. High values for .beta.-galactosidase were seen in mucopolysaccharidosis types I and II. Gaucher''s disease, juvenile amaurotic idiocy and metachromatic leucodystrophy. N-Acetyl-.beta.-glucosaminidase activity was high in mucopolysaccharidosis types I, II, III and Gaucher''s disease. The activity of .beta.-glucuronidase was increased in mucopolysaccharidosis types I, II and III, Gaucher''s disease, juvenile amaurotic idiocy and metachromatic leucodystrophy. Acid phosphatase had increased activity only in Gaucher''s disease. In several lysosomal storage disorders no increased values could be found. High values in serum from patients with lysosomal storage disorders (not including mucolipidosis II and III) may depend upon liver cell damage, which disturbs the clearing of acid hydrolases from serum.