泰-萨克斯病和桑德霍夫病中的N-乙酰基-D-葡糖胺二糖酶活性。
NN'-diacetylchitobiase activity in Tay-Sachs disease and Sandhoff's disease.
作者信息
Stirling J L
出版信息
Biochem J. 1974 Aug;141(2):597-9. doi: 10.1042/bj1410597.
Abstract
In Tay-Sachs disease and Sandhoff's disease respectively, one of the N-acetyl-beta-hexosaminidases (form A) or both (forms A and B) are absent, but glycosaminoglycans containing N-acetylhexosamines are not accumulated. The presence of NN'-diacetylchitobiase in livers from patients with these diseases raises the possibility that this new enzyme is involved in the metabolism of glycosaminoglycans rather than the enzymes previously described.
摘要
在泰-萨克斯病和桑德霍夫病中，分别缺失一种N-乙酰-β-己糖胺酶（A 型）或两种（A 型和 B 型），但含有 N-乙酰己糖胺的糖胺聚糖并未积累。这些疾病患者肝脏中存在 NN'-二乙酰壳二糖酶，这增加了一种可能性，即这种新酶参与糖胺聚糖的代谢，而非先前描述的那些酶。
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