Factor XI deficiency in a Bedouin family

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منابع مشابه

Factor XI deficiency.

That factor XI has a role in normal blood coagulation is evidenced by the fact that patients with deficiency are prone to excessive bleeding after haemostatic challenge. The role of factor XI in physiological processes has become clearer since the discovery that it is activated by thrombin; this fact has contributed to a revised model of blood coagulation. Factor XI deficiency is particularly c...

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Factor XI activity and factor XI antigen in homozygous and heterozygous factor XI deficiency.

A relatively potent antiserum against highly purified, unactivated human factor XI antigen was raised in a rabbit. This antiserum, after concentration, neutralized 50% of the factor XI clotting activity of a standard normal plasma at an antiserum dilution of 1/900. The antiserum was used in a neutralization-inhibition assay to study the relation between factor XI clotting activity and factor XI...

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Dominant Factor XI Deficiency Caused by Mutations in the Factor XI Catalytic Domain Running title: Autosomal dominant factor XI deficiency

(2497 articles) Hemostasis, Thrombosis, and Vascular Biology • (1597 articles) Free Research Articles • (3667 articles) Clinical Trials and Observations • Articles on similar topics can be found in the following Blood collections http://bloodjournal.hematologylibrary.org/site/misc/rights.xhtml#repub_requests Information about reproducing this article in parts or in its entirety may be found ...

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Three Factor 11 Mutations Associated with Factor XI Deficiency in a Turkish Family

Factor XI (FXI) is a homodimeric serine protease, which is produced in the liver and circulates in the plasma complexed with high-molecular-weight kininogen. FXI plays an important role in the amplification of the initial coagulation response via a positive feedback mechanism for the generation of additional thrombin [1,2,3,4]. Congenital FXI deficiency is characterized by decreased levels or a...

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A Classification System for Cross-Reactive Material Negative Factor XI Deficiency Running title: CRM negative factor XI deficiency

The bleeding disorder associated with factor XI (fXI) deficiency is typically inherited as an autosomal recessive trait. However, some fXI mutations may be associated with dominant disease transmission. FXI is a homodimer, a feature that could allow certain mutations to exert a dominant negative effect on wild type fXI secretion through heterodimer formation. We describe two novel fXI mutations...

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ژورنال

عنوان ژورنال: American Journal of Hematology

سال: 1996

ISSN: 0361-8609,1096-8652

DOI: 10.1002/(sici)1096-8652(199606)52:2<121::aid-ajh11>3.0.co;2-k