AMYLOID FIBRIL PROTEIN RELATED TO PRE-ALBUMIN IN FAMILIAL AMYLOIDOTIC POLYNEUROPATHY
AMYLOID FIBRIL PROTEIN RELATED TO PRE-ALBUMIN IN FAMILIAL AMYLOIDOTIC POLYNEUROPATHY
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DOI：
10.1073/pnas.75.9.4499
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发表时间：
1978-01-01
影响因子：
11.1
通讯作者：
BRAVO, FR
中科院分区：
文献类型：
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作者：
COSTA, PP;FIGUEIRA, AS;BRAVO, FR
在6例家族性淀粉样多发性神经病（FAP）患者的肾脏、甲状腺和周围神经中发现大量淀粉样纤维。将原纤维浓缩物溶解在6 M盐酸胍中，并在Sephadex G-100柱上分级。所有FAP淀粉样原纤维浓缩物的洗脱图谱显示表观Mr [分子比]为14，000的蛋白质，命名为FAP蛋白，其在通过相同程序处理的正常人体组织和原发性淀粉样变性肝的原纤维中不存在。在兔中制备的抗全变性纤维浓缩物的抗血清与FAP蛋白和正常人血清中对应于前白蛋白的组分反应。FAP蛋白与人前白蛋白具有共同的抗原决定簇，其与正常前白蛋白的同一性反应使用商业抗人前白蛋白的抗血清。用敏感的免疫化学方法不能在FAP纤维中鉴定出淀粉样AL或AA蛋白。FAP蛋白可能是FAP淀粉样纤维的独特和重要的组分，并且它与13，745 Mr前白蛋白亚基密切相关。
Amyloid fibrils were concentrated from the kidney, thyroid and peripheral nerve of 6 patients with familial amyloidotic polyneuropathy (FAP). The fibril concentrates were solubilized in 6 M guanidine.cntdot.HCl and fractionated on Sephadex G-100 columns. The elution profile of all FAP amyloid fibril concentrates revealed a protein of apparent Mr [molecular ratio] of 14,000, designated the FAP protein, that was absent from normal human tissues treated by the same procedure and from fibrils of a primary amyloidosis liver. Antisera against whole denatured fibril concentrates prepared in rabbits reacted with the FAP protein and a component in normal human serum corresponding to prealbumin. The FAP protein shared common antigenic determinants with human prealbumin by its reaction of identity with normal prealbumin using commercial antisera against human prealbumin. Amyloid AL or AA proteins could not be identified in FAP fibrils by sensitive immunochemical assay methods. The FAP protein may be a unique and significant component of the FAP amyloid fibrils and that it is closely related to the 13,745 Mr prealbumin subunit.