患有黏多糖贮积症IV型的两姐妹肝脏中N-乙酰半乳糖胺-6-硫酸酯酶的活性。
Activities of N-acetylgalactosamine-6-sulfate sulfatase in liver from two sisters with morquio syndrome.
作者信息
Minami R, Abo K, Kudoh T, Tsugawa S, Oyanagi K, Nakao T
出版信息
Tohoku J Exp Med. 1980 May;131(1):53-7. doi: 10.1620/tjem.131.53.
PMID:6773185
Abstract
A 6-sulfated tetrasaccharide obtained by digesting chondroitin-6-sulfate with testicular hyaluronidase was used as a substrate for the determination of N-acetylgalactosamine-6-sulfate sulfatase activity. The activity was not detected in liver obtained from the elder sister with clinically classic Morquio syndrome and 4.7% of the control in liver from the younger sister with the same disease.
摘要
用睾丸透明质酸酶消化硫酸软骨素-6-硫酸酯得到的一种6-硫酸化四糖用作测定N-乙酰半乳糖胺-6-硫酸酯硫酸酯酶活性的底物。在患有临床典型莫尔基奥综合征的姐姐的肝脏中未检测到该活性，而在患有相同疾病的妹妹的肝脏中检测到的活性为对照的4.7%。
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