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家族性淀粉样多神经病患者全身器官中淀粉样原纤维蛋白的定量分析。

Quantitative analysis of amyloid fibril protein in systemic organs of patients with familial amyloidotic polyneuropathy.

作者信息

Nakazato M, Tanaka M, Matsukura S, Kangawa K, Matsuo H

机构信息

Third Department of Internal Medicine, Miyazaki Medical College, Japan.

出版信息

J Neurol Sci. 1989 Feb;89(2-3):235-42. doi: 10.1016/0022-510x(89)90025-7.

DOI:10.1016/0022-510x(89)90025-7
PMID:2926451
Abstract

The purpose of this study was to develop a quantitative method for variant transthyretin (also called prealbumin) in amyloid-laden tissues of familial amyloidotic polyneuropathy (FAP). The variant transthyretin was extracted by using 70% formic acid solution in which the protein was solubilized completely and rapidly. The quantity of the variant transthyretin was measured by a radioimmunoassay (RIA). An immunoreactive species detected in the tissues of FAP was confirmed to be the variant transthyretin by reverse-phase high-performance liquid chromatography. The quantity of the variant transthyretin in the thyroid gland of 2 FAP patients was 9.90 and 9.76 micrograms/mg tissue, and that of the kidney was 4.03 and 6.27 micrograms/mg tissue, respectively, while its quantity in the liver was only 0.15 and 0.12 microgram/mg tissue. We present here a quantitative analysis of the distribution and predominance of amyloid deposits in systemic organs of FAP.

摘要

本研究的目的是开发一种定量方法,用于检测家族性淀粉样多神经病(FAP)淀粉样变组织中的变异甲状腺素运载蛋白(也称为前白蛋白)。采用70%甲酸溶液提取变异甲状腺素运载蛋白,该溶液能使蛋白质迅速完全溶解。通过放射免疫分析(RIA)测定变异甲状腺素运载蛋白的含量。通过反相高效液相色谱法证实,在FAP组织中检测到的一种免疫反应性物质为变异甲状腺素运载蛋白。两名FAP患者甲状腺组织中变异甲状腺素运载蛋白的含量分别为9.90和9.76微克/毫克组织,肾脏中分别为4.03和6.27微克/毫克组织,而肝脏中仅为0.15和0.12微克/毫克组织。我们在此展示了FAP全身器官中淀粉样沉积物分布和优势的定量分析。

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