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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。

The Hurler syndrome: treatment of cultured Hurler fibroblasts with normal human serum.

作者信息

Herd J K, Hayhome B, Tschida J

出版信息

Proc Soc Exp Biol Med. 1975 Oct;150(1):194-201. doi: 10.3181/00379727-150-39001.

DOI:10.3181/00379727-150-39001
PMID:127180
Abstract

Prolonged replacement of fetal calf serum by normal human serum for the enrichment of medium during tissue culture of Hurler fibroblasts resulted in increased acid mucopolysaccharides in the cells and in the medium. The predominant intracellular mucopolysaccharide had the characteristics of dermatan sulfate when Hurler cells were treated with either serum. Normal human serum contains a nonspecific coreective factor capable of augmenting the loss of 35SO4-AMPS from Hurler cells, but not from normal cells. Fetal calf serum and Hurler serum have similar corrective factor activity for labeled Hurler cells. The corrective factor activity of all three sera was recovered from reconstituted dialyzed ammonium sulfate precipitates. The corrective factor of normal human serum did not increase degradation of mucopolysaccharide, but increased secretion of macromolecular and large oligosaccharide components. Failure of the corrective factor of normal human serum to effectively decrease the dermatan sulfate content of Hurler cells during prolonged exposure may be a quantitative phenomenon due partly to the brief duration of corrective factor activity and partly to increased synthesis of mucopolysaccharide.

摘要

在对黏多糖贮积症Ⅰ型成纤维细胞进行组织培养时,用正常人血清长期替代胎牛血清以富集培养基,结果导致细胞和培养基中的酸性黏多糖增加。当用任何一种血清处理黏多糖贮积症Ⅰ型细胞时,细胞内主要的黏多糖具有硫酸皮肤素的特征。正常人血清含有一种非特异性矫正因子,它能够增加黏多糖贮积症Ⅰ型细胞中35SO4-AMPS的丢失,但对正常细胞无此作用。胎牛血清和黏多糖贮积症Ⅰ型血清对标记的黏多糖贮积症Ⅰ型细胞具有相似的矫正因子活性。所有三种血清的矫正因子活性均可从重构的透析硫酸铵沉淀物中恢复。正常人血清的矫正因子不会增加黏多糖的降解,但会增加大分子和大寡糖成分的分泌。正常人血清的矫正因子在长期暴露期间未能有效降低黏多糖贮积症Ⅰ型细胞中硫酸皮肤素的含量,这可能是一种定量现象,部分原因是矫正因子活性持续时间短暂,部分原因是黏多糖合成增加。

相似文献

1
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.
2
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.
3
The Hurler syndrome: a study of cultured lymphoid cell lines.黏多糖贮积症Ⅰ型:对培养的淋巴细胞系的一项研究。
J Exp Med. 1972 Sep 1;136(3):644-9. doi: 10.1084/jem.136.3.644.
4
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.
5
Biochemical studies on the sulphated glycosaminoglycan fraction of skin fibroblasts cultured from a patient with the Hurler syndrome.对一名患有Hurler综合征患者培养的皮肤成纤维细胞硫酸化糖胺聚糖部分的生化研究。
Biochem J. 1973 Mar;132(3):395-402. doi: 10.1042/bj1320395.
6
The disorder of hyaluronic acid metabolism in cultured skin fibroblasts derived from a patient with the Hurler syndrome.来自一名黏多糖贮积症I型患者的培养皮肤成纤维细胞中透明质酸代谢紊乱。
Biochem J. 1973 Mar;132(3):403-8. doi: 10.1042/bj1320403.
7
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.
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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.
9
The tissue culture detection of corrective factor activity for Hurler fibroblasts.对Hurler成纤维细胞校正因子活性的组织培养检测
Proc Soc Exp Biol Med. 1973 Jun;143(2):446-52. doi: 10.3181/00379727-143-37340.
10
Impaired elastogenesis in Hurler disease: dermatan sulfate accumulation linked to deficiency in elastin-binding protein and elastic fiber assembly.黏多糖贮积症I型中弹性蛋白生成受损:硫酸皮肤素积累与弹性蛋白结合蛋白缺乏及弹性纤维组装有关。
Am J Pathol. 2000 Mar;156(3):925-38. doi: 10.1016/S0002-9440(10)64961-9.