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用牛睾丸透明质酸酶对黏多糖贮积症I型成纤维细胞进行体外校正

In vitro correction of Hurler fibroblasts with bovine testicular hyaluronidase.

作者信息

Herd J K, Hayhome B A, Tschida J, Forrest T

出版信息

Proc Soc Exp Biol Med. 1976 Apr;151(4):642-9. doi: 10.3181/00379727-151-39277.

DOI:10.3181/00379727-151-39277
PMID:131320
Abstract

Bovine testicular hyaluronidase (endo-beta-N-acetyl hexosaminidase) has a significant corrective effect on cultured Hurler fibroblasts. Nonspecificity of this effect is indicated by its equally strong corrective effect on Hunter fibroblasts. Although all specimens of hyaluronidase also possessed iduronidase activity, a separate corrective effect could be attributed to the endo-N-acetyl hexosaminidase activity of at least one hyaluronidase (Wyeth M-151) for four reasons: (i) its very low content of iduronidase activity; (ii) a decrease in intracellular macromolecular mucopolysaccharides (believed to be largely dermatan sulfate) with a corresponding increase in intracellular and extracellular oligosaccharides; (iii) no measurable increase in iduronidase activity of hyaluronidase-treated cells despite near maximal correction; (iv) direct correlation between Hurler cell correction and hyaluronidase activity when enzymes of different strength were used at less than maximal correction.

摘要

牛睾丸透明质酸酶(内切-β-N-乙酰己糖胺酶)对培养的Hurler成纤维细胞有显著的校正作用。这种作用的非特异性体现在它对Hunter成纤维细胞也有同样强的校正作用。尽管所有透明质酸酶标本也都具有艾杜糖醛酸酶活性,但至少有一种透明质酸酶(惠氏M-151)的内切-N-乙酰己糖胺酶活性可归因于一种单独的校正作用,原因有四点:(i)其艾杜糖醛酸酶活性含量极低;(ii)细胞内大分子粘多糖(认为主要是硫酸皮肤素)减少,同时细胞内和细胞外寡糖相应增加;(iii)尽管校正接近最大值,但透明质酸酶处理的细胞中艾杜糖醛酸酶活性没有可测量的增加;(iv)当使用不同强度的酶且校正未达到最大值时,Hurler细胞校正与透明质酸酶活性之间存在直接相关性。

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1
In vitro correction of Hurler fibroblasts with bovine testicular hyaluronidase.用牛睾丸透明质酸酶对黏多糖贮积症I型成纤维细胞进行体外校正
Proc Soc Exp Biol Med. 1976 Apr;151(4):642-9. doi: 10.3181/00379727-151-39277.
2
Hurler and Hunter syndromes: mutual correction of the defect in cultured fibroblasts.黏多糖贮积症Ⅰ型和Ⅱ型:培养成纤维细胞中缺陷的相互校正。
Science. 1968 Nov 1;162(3853):570-2. doi: 10.1126/science.162.3853.570.
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The Hurler syndrome: treatment of cultured Hurler fibroblasts with normal human serum.黏多糖贮积症Ⅰ型:用人正常血清培养黏多糖贮积症Ⅰ型成纤维细胞的治疗方法。
Proc Soc Exp Biol Med. 1975 Oct;150(1):194-201. doi: 10.3181/00379727-150-39001.
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The defect in Hurler and Hunter syndromes. II. Deficiency of specific factors involved in mucopolysaccharide degradation.黏多糖贮积症Ⅰ型和Ⅱ型中的缺陷。Ⅱ. 黏多糖降解中特定因子的缺乏。
Proc Natl Acad Sci U S A. 1969 Sep;64(1):360-6. doi: 10.1073/pnas.64.1.360.
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Glycosaminoglycan degradation fragments in mucopolysaccharidosis I.黏多糖贮积症I型中的糖胺聚糖降解片段
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Comparative structural studies of urinary glycosaminoglycans in the Hurler and Hunter syndromes.黏多糖贮积症Ⅰ型和Ⅱ型中尿糖胺聚糖的比较结构研究。
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The Hurler and Hunter syndromes.黏多糖贮积症Ⅰ型和Ⅱ型综合征。
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Chimeric glycosaminoglycan oligosaccharides synthesized by enzymatic reconstruction and their use in substrate specificity determination of Streptococcus hyaluronidase.通过酶促重构合成的嵌合糖胺聚糖寡糖及其在透明质酸酶底物特异性测定中的应用。
J Biochem. 2000 Apr;127(4):695-702. doi: 10.1093/oxfordjournals.jbchem.a022659.
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Characterization of dermatan sulfate in mucopolysaccharidosis VI. Evidence for the absence of hyaluronidase-like enzymes in human skin fibroblasts.黏多糖贮积症VI型中硫酸皮肤素的特征。人类皮肤成纤维细胞中缺乏类透明质酸酶的证据。
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Accumulation of sulfate-containing acid mucopolysaccharides in I-cell fibroblasts.含硫酸酸性黏多糖在I型细胞成纤维细胞中的蓄积。
J Lab Clin Med. 1975 Oct;86(4):672-82.