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Fibrinogen Lima. A new dysfibrinogenaemia with a high-molecular-weight alpha-chain and effective polymerization.

作者信息

Arocha-Piñango C L, Rodriguez S, Nagy H, Perez Requejo J L

机构信息

Instituto Venezolano de Investigaciones Científicas, Caracas, Venezuela.

出版信息

Blood Coagul Fibrinolysis. 1990 Oct;1(4-5):561-5. doi: 10.1097/00001721-199010000-00040.

DOI:10.1097/00001721-199010000-00040
PMID:2133234
Abstract

A patient with transient microhaematuria was studied. Coagulation tests revealed prolonged thrombin and reptilase times concomitant with abnormal fibrin polymerization rate (also abnormal in both parents). In the patient and her patients, the abnormal fibrin polymerization rate was only slightly corrected by addition of calcium ions. The alpha-chain had a molecular weight higher than normal and there was deficient formation of alpha-polymers. The molecule showed a more anodal migration than the control. The abnormality described has been classified as Fibrinogen Lima.

摘要

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引用本文的文献

1
Fibrinogen Lima: a homozygous dysfibrinogen with an A alpha-arginine-141 to serine substitution associated with extra N-glycosylation at A alpha-asparagine-139. Impaired fibrin gel formation but normal fibrin-facilitated plasminogen activation catalyzed by tissue-type plasminogen activator.利马纤维蛋白原：一种纯合子异常纤维蛋白原，具有Aα-精氨酸141至丝氨酸的替换，与Aα-天冬酰胺139处额外的N-糖基化相关。纤维蛋白凝胶形成受损，但组织型纤溶酶原激活剂催化的纤维蛋白促进的纤溶酶原激活正常。
J Clin Invest. 1992 Jul;90(1):67-76. doi: 10.1172/JCI115857.