神经元蜡样脂褐质沉积症患者大脑中的β蛋白免疫反应性：超微结构和生化证明

Beta-protein immunoreactivity in brains of patients with neuronal ceroid lipofuscinosis: ultrastructural and biochemical demonstration.

作者信息

Kitaguchi T, Wisniewski K E, Maslinski S, Maslinska D, Wisniewski T M, Kim K S

机构信息

New York State Office of Mental Retardation and Developmental Disabilities, Department of Pathological Neurobiology, State Island 10314.

出版信息

Neurosci Lett. 1990 May 4;112(2-3):155-60. doi: 10.1016/0304-3940(90)90195-f.

DOI:10.1016/0304-3940(90)90195-f

PMID:2193254

Abstract

The storage pigment in neuronal ceroid lipofuscinoses (NCL) has a close similarity to age pigment lipofuscin. We studied immunoreactivity of isolated neuronal pigments from the juvenile form of NCL and aging control, using monoclonal antibodies (mAbs) against amyloid beta-protein. Ultrastructural localization of the immunoreactivity demonstrated that in NCL the epitopes are distributed mainly in curvilinear multilamellar arrays of the storage pigments and less in fingerprint profiles, while in aging control they are more homogeneously distributed on age pigment lipofuscin. The different distribution of the epitopes may reflect some catabolic as well as morphologic differences in lysosomes. A unique 31-kDa polypeptide detected on Western blots in NCL possibly derives from the same precursor, amyloid beta-protein precursor (ABPP). ABPP processing may be aberrant in NCL brains, and this can be detected as a 31-kDa polypeptide reactive with the mAbs.

摘要

神经元蜡样脂褐质沉积症（NCL）中的储存色素与老年色素脂褐质极为相似。我们使用抗β-淀粉样蛋白的单克隆抗体（mAb），研究了从青少年型NCL和衰老对照中分离出的神经元色素的免疫反应性。免疫反应性的超微结构定位表明，在NCL中，表位主要分布在储存色素的曲线状多层膜结构中，在指纹状结构中分布较少，而在衰老对照中，它们在老年色素脂褐质上分布更为均匀。表位的不同分布可能反映了溶酶体在分解代谢以及形态学上的一些差异。在NCL的蛋白质印迹法中检测到的一种独特的31 kDa多肽可能来源于相同的前体，即β-淀粉样蛋白前体（ABPP）。ABPP的加工过程在NCL大脑中可能异常，这可以通过与mAb反应的31 kDa多肽检测到。