{"title":"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。","authors":"K von Figura, M Lögering, H Kresse","doi":"10.1515/cclm.1975.13.7.285","DOIUrl":null,"url":null,"abstract":"<p><p>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.</p>","PeriodicalId":23822,"journal":{"name":"Zeitschrift fur klinische Chemie und klinische Biochemie","volume":"13 7","pages":"285-9"},"PeriodicalIF":0.0000,"publicationDate":"1975-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1515/cclm.1975.13.7.285","citationCount":"5","resultStr":"{\"title\":\"Serum alpha-N-acetylglucosaminidase: determination, characterization, and corrective activity in Sanifilippo B fibroblasts.\",\"authors\":\"K von Figura, M Lögering, H Kresse\",\"doi\":\"10.1515/cclm.1975.13.7.285\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>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.</p>\",\"PeriodicalId\":23822,\"journal\":{\"name\":\"Zeitschrift fur klinische Chemie und klinische Biochemie\",\"volume\":\"13 7\",\"pages\":\"285-9\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"1975-07-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://sci-hub-pdf.com/10.1515/cclm.1975.13.7.285\",\"citationCount\":\"5\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Zeitschrift fur klinische Chemie und klinische Biochemie\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1515/cclm.1975.13.7.285\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Zeitschrift fur klinische Chemie und klinische Biochemie","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1515/cclm.1975.13.7.285","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
引用次数: 5
摘要
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。
Serum alpha-N-acetylglucosaminidase: determination, characterization, and corrective activity in Sanifilippo B fibroblasts.
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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