Serum alpha-N-acetylglucosaminidase: determination, characterization, and corrective activity in Sanifilippo B fibroblasts.

Zeitschrift fur klinische Chemie und klinische Biochemie Pub Date : 1975-07-01 DOI:10.1515/cclm.1975.13.7.285

K von Figura, M Lögering, H Kresse

引用次数: 5

Abstract

Assays for the determination of serum alpha-N-acetylglucosaminidase (EC 3.2.1.50) activity are described employing p-nitrophenyl-N-acetyl-alpha-D-glucosaminide, phenyl-N-acetyl-alpha-D-glucosaminide, and UDP-N-acetylglucosamine as substrates. A log normal distribution of the serum enzyme activity was found. The determination of serum alpha-N-acetylglucosaminidase activity proved to be a valuable tool for the recognition of homozygous and heterozygous carriers of the Sanfilippo B gene.

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血清α - n -乙酰氨基葡萄糖酶:Sanifilippo B成纤维细胞的测定、表征和纠正活性。

测定血清α -n -乙酰氨基葡萄糖酶(EC 3.2.1.50)活性的方法采用对硝基苯基-n -乙酰基- α - d -氨基葡萄糖、苯基-n -乙酰基- α - d -氨基葡萄糖和udp -n -乙酰氨基葡萄糖作为底物。血清酶活性呈对数正态分布。血清α - n -乙酰氨基葡萄糖酶活性的测定被证明是识别Sanfilippo B基因纯合子和杂合子携带者的有价值的工具。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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Zeitschrift fur klinische Chemie und klinische Biochemie

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