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神经元蜡样脂褐质沉积症的研究:神经元自发荧光脂褐素的异质性本质

Studies in neuronal ceroid-lipofuscinosis: heterogeneous nature of neuronal autofluorescent lipopigments.

作者信息

Garg H S, Awasthi Y C, Srivastava S K

出版信息

J Neurosci Res. 1981;6(6):771-83. doi: 10.1002/jnr.490060611.

DOI:10.1002/jnr.490060611
PMID:7334535
Abstract

A method has been developed for the complete extraction and fractionation of the autofluorescent lipopigments present in the neuronal tissue of Batten-Spielmeyer-Vogt (BSV) patient and normal subject, respectively. The fluorescent lipopigments that were about three-fold higher in BSV patient's brain as compared with normal subjects were separated into at least 7 fluorometrically and chromatographically distinct fractions by thin layer chromatography (TLC). Although the fluorescent lipopigments of the brain tissue of BSV patient and normal subject showed identical pattern of separation on TLC, the flourescence spectra of the individual lipopigment fractions from BSV patient was different from those of the corresponding fractions from the normal subject. The ultraviolet spectral studies with individual fluorescent lipopigment fractions indicated the presence of highly unsaturated conjugated chromophore.

摘要

已经开发出一种方法,分别用于从巴顿-施皮勒迈尔-沃格特(BSV)患者和正常受试者的神经元组织中完全提取和分离自发荧光脂色素。与正常受试者相比,BSV患者大脑中荧光脂色素含量高出约三倍,通过薄层色谱法(TLC)将其分离为至少7个在荧光和色谱上不同的组分。尽管BSV患者和正常受试者脑组织的荧光脂色素在TLC上显示出相同的分离模式,但BSV患者单个脂色素组分的荧光光谱与正常受试者相应组分的荧光光谱不同。对单个荧光脂色素组分的紫外光谱研究表明存在高度不饱和的共轭发色团。

相似文献

1
Studies in neuronal ceroid-lipofuscinosis: heterogeneous nature of neuronal autofluorescent lipopigments.神经元蜡样脂褐质沉积症的研究:神经元自发荧光脂褐素的异质性本质
J Neurosci Res. 1981;6(6):771-83. doi: 10.1002/jnr.490060611.
2
[Acute optical hallucinations in the juvenile neuronal ceroid lipofuscinosis (Spielmeyer-Vogt syndrome)].[青少年神经元蜡样脂褐质沉积症(斯皮尔曼-沃格特综合征)中的急性视幻觉]
Acta Paedopsychiatr. 1981 May;47(1):19-25.
3
[Infantile form of S.C. neuronal ceroid - lipofuscinosis (author's transl)].婴儿型神经元蜡样脂褐质沉积症(作者译)
Sb Lek. 1976;78(11-12NA-NA-770124-770126):316-9.
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Is neuronal ceroid lipofuscinosis a single disease entity?神经元蜡样脂褐质沉积症是一种单一的疾病实体吗?
Trans Am Neurol Assoc. 1975;100:227-9.
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Atypical neuronal ceroid-lipofuscinosis.非典型神经元蜡样脂褐质沉积症
Neurology. 1978 Jul;28(7):710-7. doi: 10.1212/wnl.28.7.710.
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The neurophysiological features of various diseases with NCL storage.各种伴有NCL蓄积疾病的神经生理学特征。
Riv Patol Nerv Ment. 1976 Aug;97(4):186-90.
7
Electron microscopy of skin and peripheral blood lymphocytes in infantile (Santavuori) neuronal ceroid lipofuscinosis.婴儿型(桑塔武奥里型)神经元蜡样脂褐质沉积症皮肤及外周血淋巴细胞的电子显微镜检查
Neuropadiatrie. 1979 Aug;10(3):245-63. doi: 10.1055/s-0028-1085328.
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[Batten's disease (neuronal ceroid-lipofuscinosis): diagnosis of a Janský-Bielschowsky type by ultrastructural study of a skin biopsy. Nosologic status and general review].[巴顿病(神经元蜡样脂褐质沉积症):通过皮肤活检的超微结构研究诊断为扬斯基-比尔绍夫斯基型。疾病分类状况及综述]
Union Med Can. 1978 Jun;107(6):535-42, 613.
9
Lipofuscin in neuronal aging and diseases.神经元衰老和疾病中的脂褐素。
Norm Pathol Anat (Stuttg). 1976;32:1-68.
10
[Characteristics of the composition of fluorescent lipopigments of the liver and brain in rats of various ages].
Fiziol Zh (1978). 1989 Sep-Oct;35(5):37-42.

引用本文的文献

1
Neuronal ceroid lipofuscinosis. Ocular histopathologic and electron microscopic studies in the late infantile, juvenile, and adult forms.神经元蜡样脂褐质沉积症。晚期婴儿型、青少年型和成人型的眼部组织病理学及电子显微镜研究。
Graefes Arch Clin Exp Ophthalmol. 1987;225(6):391-402. doi: 10.1007/BF02334164.