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遗传性淀粉样变性伴格子状角膜营养不良及继发性淀粉样变性中淀粉样蛋白的部分特征分析

Partial characterization of amyloid proteins in inherited amyloidosis with lattice corneal dystrophy and in secondary amyloidosis.

作者信息

Meretoja J, Hollmén T, Meretoja T, Penttinen R

出版信息

Med Biol. 1978 Feb;56(1):17-22.

PMID:305513
Abstract

Amyloid fibrils isolated from two patients, one with inherited systemic amyloidosis and lattice corneal dystrophy, and the other with secondary amyloidosis due to chronic glomerulonephritis, were studied using immunologic, electrophoretic and chromatographic techniques. Amino acid hydrolysates of both amyloid types showed a high proportion of acidic and aliphatic amino acid residues but were non-identical in the overall amino acid composition. The preparations also contained 12--16% lipids. Guanidine hydrochloride denaturated amyloid fibrils of both types were resolved into four fractions in Sepharose 6B chromatography with molecular weights of ca. 160 000, 45 000, 20 000 and 8 000. The 160 000 mol. wt. fraction predominated in the chromatograms of inherited amyloid protein and was further resolved into two main fractions of 17 000 and 15 000 mol. wt. in sodium dodecyl sulfate (SDS) polyacrylamide gel electrophoresis. None of these fractions from inherited amyloid protein showed immunologic identity with tissue-derived amyloid protein A (AA) whereas all four fractions from secondary amyloid reacted against anti-AA antiserum. The three major Sepharose 6B fractions of secondary amyloid fibrils were resolved into a 25 000 mol. wt. fraction in SDS-polyacrylamide gel electrophoresis without urea but into a 12 000 mol. wt. fraction in gels containing 8M urea after more drastic dissolving conditions of the fibrils.

摘要

从两名患者身上分离出淀粉样纤维进行研究,一名患者患有遗传性全身性淀粉样变性和格子状角膜营养不良,另一名患者因慢性肾小球肾炎患有继发性淀粉样变性,研究采用了免疫、电泳和色谱技术。两种淀粉样蛋白的氨基酸水解产物均显示出高比例的酸性和脂肪族氨基酸残基,但整体氨基酸组成并不相同。制剂中还含有12%至16%的脂质。在琼脂糖6B色谱中,两种类型的盐酸胍变性淀粉样纤维均被分离为四个组分,分子量约为160000、45000、20000和8000。160000分子量的组分在遗传性淀粉样蛋白的色谱图中占主导地位,在十二烷基硫酸钠(SDS)聚丙烯酰胺凝胶电泳中进一步分离为17000和15000分子量的两个主要组分。遗传性淀粉样蛋白的这些组分均未显示与组织来源的淀粉样蛋白A(AA)有免疫同一性,而继发性淀粉样蛋白的所有四个组分均与抗AA抗血清发生反应。继发性淀粉样纤维的三个主要琼脂糖6B组分在不含尿素的SDS聚丙烯酰胺凝胶电泳中分离为25000分子量的组分,但在对纤维进行更剧烈溶解条件后,在含8M尿素的凝胶中分离为12000分子量的组分。

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Partial characterization of amyloid proteins in inherited amyloidosis with lattice corneal dystrophy and in secondary amyloidosis.遗传性淀粉样变性伴格子状角膜营养不良及继发性淀粉样变性中淀粉样蛋白的部分特征分析
Med Biol. 1978 Feb;56(1):17-22.
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引用本文的文献

1
Amyloid in familial amyloidosis, Finnish type, is antigenically and structurally related to gelsolin.芬兰型家族性淀粉样变性中的淀粉样蛋白在抗原性和结构上与凝溶胶蛋白相关。
Am J Pathol. 1990 Jun;136(6):1223-8.
2
Gelsolin-related amyloidosis. Identification of the amyloid protein in Finnish hereditary amyloidosis as a fragment of variant gelsolin.凝溶胶蛋白相关淀粉样变性。芬兰遗传性淀粉样变性中淀粉样蛋白被鉴定为变异型凝溶胶蛋白的一个片段。
J Clin Invest. 1991 Apr;87(4):1195-9. doi: 10.1172/JCI115118.