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关于黏多糖贮积症Ⅰ型中肝脏酸性糖胺聚糖的进一步研究。

Further studies on hepatic acidic glycosaminoglycans in the Hurler syndrome.

作者信息

Kofoed J A, Tocci A A, Barceló A C

出版信息

Experientia. 1978 May 15;34(5):577-8. doi: 10.1007/BF01936968.

DOI:10.1007/BF01936968
PMID:95964
Abstract

Acid GAG were isolated from hepatic tissue from 3 patients with Hurler syndrome and 3 normal controls. Gross elevations in the uronic acid and hexosamine contents were found in Hurler livers compared with the normal ones. The total GAG concentration was significantly increased (about 25fold) in Hurler patients.

摘要

从3例黏多糖贮积症I型患者及3名正常对照者的肝脏组织中分离出酸性糖胺聚糖。与正常肝脏相比,黏多糖贮积症I型患者肝脏中的糖醛酸和氨基己糖含量显著升高。黏多糖贮积症I型患者的总糖胺聚糖浓度显著增加(约25倍)。

相似文献

1
Further studies on hepatic acidic glycosaminoglycans in the Hurler syndrome.关于黏多糖贮积症Ⅰ型中肝脏酸性糖胺聚糖的进一步研究。
Experientia. 1978 May 15;34(5):577-8. doi: 10.1007/BF01936968.
2
Oversulfated dermatan sulfate extracted from Hurler spleen.
Anal Biochem. 1968 Oct 24;25(1):370-8. doi: 10.1016/0003-2697(68)90111-5.
3
Hepatic acid mucopolysaccharides in the mucopolysaccharidoses type I, II and 3.
Clin Biochem. 1971 Oct;4(3):147-55. doi: 10.1016/s0009-9120(71)91222-7.
4
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.
5
The biochemistry of Hurler's syndrome.黏多糖贮积症Ⅰ型的生物化学
Enzymol Biol Clin (Basel). 1969;10(6):534-48. doi: 10.1159/000458339.
6
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.
7
Acidic glycosaminoglycans and gangliosides in the brains from four patients with genetic mucopolysaccharidosis.
Tohoku J Exp Med. 1982 Jul;137(3):253-60. doi: 10.1620/tjem.137.253.
8
The Hurler and Hunter syndromes.黏多糖贮积症Ⅰ型和Ⅱ型综合征。
Am J Med. 1969 Nov;47(5):691-707. doi: 10.1016/0002-9343(69)90164-8.
9
Structural studies on heparitin sulfate of normal and Hurler tissues.
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Acidic glycosaminoglycans in liver from five patients with mucopolysaccharidosis and mucolipidosis.
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本文引用的文献

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DISTINCTION AMONG FOUR FORMS OF HURLER'S SYNDROME.黏多糖贮积症Ⅰ型四种形式的鉴别
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L-iduronidase in cultured human fibroblasts and liver.培养的人成纤维细胞和肝脏中的艾杜糖醛酸酶
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Gargoylism (mucopolysaccharidosis type II). Accumulation of glycosaminoglycans, gangliosides and glycoproteins and activity of some related glycolytic enzymes in liver, spleen and brain.黏多糖贮积症Ⅱ型(承雷氏病)。肝脏、脾脏和大脑中糖胺聚糖、神经节苷脂和糖蛋白的蓄积以及一些相关糖酵解酶的活性。
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Hurler's syndrome, an -L-iduronidase deficiency.胡勒综合征,一种α-L-艾杜糖醛酸酶缺乏症。
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Hepatic acid mucopolysaccharides in the mucopolysaccharidoses type I, II and 3.
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