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Fibrinogen Columbus: a novel gamma Gly200Val mutation causing hypofibrinogenemia in a family with associated thrombophilia.
Haematologica ; 92(8): 1151-2, 2007 Aug.
Article en En | MEDLINE | ID: mdl-17650452
Fibrinogen is an essential component of the coagulation cascade and the acute phase response. The native 340 kDa molecule has a symmetrical trinodular structure composed of a central E-domain connected to outer D-domains by triple helical coiled-coils.1 Several mutations known to cause hypofibrinogenemia occur within the C-terminal gammaD-domain and have helped to elucidate the structurally and functionally important areas of this domain.2-5 Here we report the identification of a novel point mutation gammaG200V (fibrinogen Columbus) causing hypofibrinogenemia and co-segregating with three genetic thrombophilia risk factors.
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Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Fibrinógenos Anormales / Mutación Puntual / Trombofilia / Sustitución de Aminoácidos / Resistencia a la Proteína C Activada / Mutación Missense / Afibrinogenemia / Metilenotetrahidrofolato Reductasa (NADPH2) Tipo de estudio: Etiology_studies / Prognostic_studies / Risk_factors_studies Límite: Adult / Child, preschool / Female / Humans / Male / Newborn Idioma: En Revista: Haematologica Año: 2007 Tipo del documento: Article Pais de publicación: Italia
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Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Fibrinógenos Anormales / Mutación Puntual / Trombofilia / Sustitución de Aminoácidos / Resistencia a la Proteína C Activada / Mutación Missense / Afibrinogenemia / Metilenotetrahidrofolato Reductasa (NADPH2) Tipo de estudio: Etiology_studies / Prognostic_studies / Risk_factors_studies Límite: Adult / Child, preschool / Female / Humans / Male / Newborn Idioma: En Revista: Haematologica Año: 2007 Tipo del documento: Article Pais de publicación: Italia