Westermark P, Natvig J B, Johansson B
J Exp Med. 1977 Aug 1;146(2):631-6. doi: 10.1084/jem.146.2.631.
A protein, ASCA, is isolated from amyloid fibrils extracted from heart tissue of five different patients with senile cardiac amyloidosis (SCA). The proteins of all five patients showed immunological identity when reacted with an antiserum raised against one of the proteins. In contrast, no reaction was obtained with antisera against a variety of other amyloid proteins. The antiserum against the subunit protein of senile cardiac amyloid did not react with any other amyloid preparations tested, nor with extracts of normal heart tissue. Thus, the subunit protein appeared to be unique to senile heart amyloid. The protein could form fibrils in vitro, had a mol wt of about 6,000 daltons and the amino acid compositions investigated in two cases showed extensive similarities but were clearly different from that of protein AA of secondary amyloid fibrils.
从五名不同的老年心脏淀粉样变性(SCA)患者心脏组织中提取的淀粉样纤维中分离出一种蛋白质,即ASCA。当这五种蛋白质与针对其中一种蛋白质产生的抗血清反应时,它们显示出免疫同一性。相比之下,针对多种其他淀粉样蛋白的抗血清未产生反应。针对老年心脏淀粉样变性亚基蛋白的抗血清与测试的任何其他淀粉样制剂均无反应,也与正常心脏组织提取物无反应。因此,该亚基蛋白似乎是老年心脏淀粉样变性所特有的。该蛋白质在体外可形成纤维,分子量约为6000道尔顿,在两个案例中研究的氨基酸组成显示出广泛的相似性,但明显不同于继发性淀粉样纤维的AA蛋白。