Mucopolysaccharidosis in a cat with arylsulfatase B deficiency: a model of Maroteaux-Lamy syndrome
Mucopolysaccharidosis in a cat with arylsulfatase B deficiency: a model of Maroteaux-Lamy syndrome
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芳基硫酸酯酶 B 缺乏症猫的粘多糖贮积症：Maroteaux-Lamy 综合征模型
作者：
Jezyk Pf;M. Haskins;D. Patterson;W. Mellman;M. Greenstein
研究了一只暹罗猫，其临床体征与在患有粘多糖沉积症的人类中观察到的相似。该动物在尿液中排泄的聚合糖胺聚糖量增加，几乎完全由硫酸皮肤素组成。循环多形核白细胞的电子显微镜显示存在许多膜结合的层状包涵体。培养的皮肤成纤维细胞的硫酸盐掺入研究表明糖胺聚糖降解缺陷。这些细胞显示芳基硫酸酯酶B活性缺乏。这种疾病似乎与人类中描述的Maroteaux-Lamy综合征相似或相同。
A Siamese cat that presented clinical signs similar to those seen in humans with mucopolysaccharidoses was studied. The animal excreted increased amounts of polymeric glycosaminoglycans in the urine, consisting almost entirely of dermatan sulfate. Electron microscopy of circulating polymorphonuclear leukocytes revealed the presence of many membrane-bound lamellar inclusion bodies. Sulfate incorporation studies with cultured skin fibroblasts indicated defective glycosaminoglycan degradation. These cells showed a deficiency in arylsulfatase B activity. The disorder appears similar or identical to the Maroteaux-Lamy syndrome described in humans.