The accumulation of cerebroside sulfates by fibroblasts in culture from patients with late infantile metachromatic leukocystrophy.

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作者信息

Porter M T, Fluharty A L, Harris S E, Kihara H

出版信息

Arch Biochem Biophys. 1970 Jun;138(2):646-52. doi: 10.1016/0003-9861(70)90392-9.

DOI:10.1016/0003-9861(70)90392-9

PMID:4194292

Abstract

摘要

相似文献

The accumulation of cerebroside sulfates by fibroblasts in culture from patients with late infantile metachromatic leukocystrophy.

Arch Biochem Biophys. 1970 Jun;138(2):646-52. doi: 10.1016/0003-9861(70)90392-9.

Correction of abnormal cerebroside sulfate metabolism in cultured metachromatic leukodystrophy fibroblasts.培养的异染性脑白质营养不良成纤维细胞中异常脑硫脂代谢的校正

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Correction of the defective sulfatide degradation in cultured fibroblasts from patients with metachromatic leucodystrophy.

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Treatment of metachromatic leukodystrophy in fibroblasts by enzyme replacement.通过酶替代疗法治疗成纤维细胞中的异染性脑白质营养不良。

N Engl J Med. 1971 Mar 25;284(12):672-3. doi: 10.1056/NEJM197103252841214.

The patterns of arylsulphatases A and B in human normal and metachromatic leucodystrophy tissues and their relationship to the cerebroside sulphatase activity.人正常组织和异染性脑白质营养不良组织中芳基硫酸酯酶A和B的模式及其与脑苷脂硫酸酯酶活性的关系。

J Neurochem. 1973 Feb;20(2):279-87. doi: 10.1111/j.1471-4159.1973.tb12127.x.

A correlation of intracellular cerebroside sulfatase activity in fibroblasts with latency in metachromatic leukodystrophy.成纤维细胞内脑苷脂硫酸酯酶活性与异染性脑白质营养不良潜伏期的相关性。

Biochem Biophys Res Commun. 1971 Aug 6;44(3):660-6. doi: 10.1016/s0006-291x(71)80134-1.

[Two types of cerebroside sulfates as so-called prelipids and storage substances in leukodystrophy of type Scholz (metachromatic form of diffuse sclerosis)].[两种脑硫脂作为所谓的前脂和施尔茨型脑白质营养不良（弥漫性硬化的异染性形式）中的储存物质]

Hoppe Seylers Z Physiol Chem. 1958;311(4-6):279-82.

Cerebroside-sulphatase and arylsulphatase A deficiency in metachromatic leukodystrophy (ML).异染性脑白质营养不良（ML）中的脑苷脂硫酸酯酶和芳基硫酸酯酶A缺乏症。

J Neurochem. 1969 Jan;16(1):19-28. doi: 10.1111/j.1471-4159.1969.tb10339.x.

[Sulfatide lipoidosis in childhood].儿童期硫酸脂类脂质沉积症

Monatsschr Kinderheilkd (1902). 1967 Apr;115(4):250-4.

Evaluation of the metabolic defect in metachromatic leukodystrophy (MLD).异染性脑白质营养不良（MLD）代谢缺陷的评估。

Trans Am Neurol Assoc. 1967;92:171-4.

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High-resolution loading tests in the study of genetic heterogeneity in gangliosidosis fibroblasts.神经节苷脂贮积症成纤维细胞遗传异质性研究中的高分辨率负荷试验

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Functional compartments of sulphatide metabolism in cultured living cells: evidence for the involvement of a novel sulphatide-degrading pathway.培养活细胞中硫脂代谢的功能区室：一种新型硫脂降解途径参与其中的证据。

Biochem J. 1994 Feb 1;297 ( Pt 3)(Pt 3):479-89. doi: 10.1042/bj2970479.

Multiple sulfatase deficiency (mucosulfatidosis): impaired degradation of labeled sulfated compounds in cultured skin fibroblasts in vivo.多种硫酸酯酶缺乏症（粘脂硫酸酯贮积症）：体内培养的皮肤成纤维细胞中标记的硫酸化化合物降解受损。

Eur J Pediatr. 1980 Oct;135(1):85-9. doi: 10.1007/BF00445900.

Diagnosis of metachromatic leukodystrophy, Krabbe disease, and Farber disease after uptake of fatty acid-labeled cerebroside sulfate into cultured skin fibroblasts.在脂肪酸标记的硫酸脑苷脂被培养的皮肤成纤维细胞摄取后，对异染性脑白质营养不良、克拉伯病和法伯病进行诊断。

J Clin Invest. 1982 Jul;70(1):89-97. doi: 10.1172/jci110607.

Atypical metachromatic leukodystrophy? Problems with the biochemical diagnosis.非典型异染性脑白质营养不良？生化诊断的问题。

Hum Genet. 1984;67(2):170-3. doi: 10.1007/BF00272994.

Genetic heterogeneity in metachromatic leukodystrophy.异染性脑白质营养不良中的遗传异质性。

Am J Hum Genet. 1982 Mar;34(2):171-81.

The AB-variant of metachromatic leukodystrophy (postulated activator protein deficiency). Light and electron microscopic findings in a sural nerve biopsy.异染性脑白质营养不良的AB变异型（推测为激活蛋白缺乏）。腓肠神经活检的光镜和电镜检查结果。

Acta Neuropathol. 1981;55(4):281-7. doi: 10.1007/BF00690991.

Investigation of degenerative disease of the central nervous system.中枢神经系统退行性疾病的研究。

Arch Dis Child. 1972 Apr;47(252):163-70. doi: 10.1136/adc.47.252.163.

Simplified procedure for preparation of 35S-labeled brain sulfatide.制备35S标记脑硫脂的简化程序

Lipids. 1974 Nov;9(11):865-9. doi: 10.1007/BF02532611.

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