纤维蛋白原札幌型：一种异常纤维蛋白原血症，其特征为αA链精氨酸-16被组氨酸取代，导致凝血酶释放纤维蛋白肽A延迟。

Fibrinogen Sapporo: dysfibrinogenemia characterized by the replacement of A alpha arginine-16 by histidine resulting in the delayed release of fibrinopeptide A by thrombin.

作者信息

Asakura S, Terukina S, Yamazumi K, Matsuda M, Murayama H, Higuchi A, Musashi M, Sakurada K, Miyazaki T

出版信息

Nihon Ketsueki Gakkai Zasshi. 1989 Sep;52(6):1094-104.

Abstract

Congenital dysfibrinogenemia was found in a 60-year-old asymptomatic female and her daughter. Purified fibrinogen derived from the propositus, apparently a heterozygote for the abnormality, characteristically showed delayed but complete release of fibrinopeptide A upon digestion with thrombin but its defective release by Ancrod, a snake venom enzyme, from half of her fibrinogen molecules. This congenital dysfibrinogenemia with an A alpha arginine (Arg) to histidine (His) substitution was tentatively designated as fibrinogen Sapporo. Although this type of abnormal fibrinogen had been identified among Caucasians, no such cases have so far been reported in Japan.

摘要

在一名60岁无症状女性及其女儿身上发现了先天性异常纤维蛋白原血症。从先证者（显然是该异常的杂合子）中纯化得到的纤维蛋白原，其特征是在用凝血酶消化后，纤维蛋白肽A的释放延迟但完全，而在用蛇毒酶安克洛消化时，其一半的纤维蛋白原分子释放存在缺陷。这种具有α-精氨酸（Arg）到组氨酸（His）替代的先天性异常纤维蛋白原血症被暂定命名为纤维蛋白原札幌型。虽然这种类型的异常纤维蛋白原在高加索人群中已被鉴定出，但迄今为止在日本尚未有此类病例的报道。