{"id":90288,"date":"2018-03-10T00:15:42","date_gmt":"2018-03-10T00:15:42","guid":{"rendered":"https:\/\/www.deberes.net\/tesis\/sin-categoria\/fisiopatologia-del-receptor-plaquetario-de-fibrinogeno\/"},"modified":"2018-03-10T00:15:42","modified_gmt":"2018-03-10T00:15:42","slug":"fisiopatologia-del-receptor-plaquetario-de-fibrinogeno","status":"publish","type":"post","link":"https:\/\/www.deberes.net\/tesis\/ciencias-medicas\/fisiopatologia-del-receptor-plaquetario-de-fibrinogeno\/","title":{"rendered":"Fisiopatolog\u00eda del receptor plaquetario de fibrinogeno"},"content":{"rendered":"<h2>Tesis doctoral de <strong> Elena Garcia Arias-salgado <\/strong><\/h2>\n<p>La tromboastenia de glanzmann es una enfermedad hereditaria caracterizada por un defecto cuantitativo o funcional del receptor plaquetario de fibrinogeno (gpiib\/iiia). Por ello, nos ha servido de modelo para ampliar los conocimientos acerca de este receptor. Hemos estudiado cinco familias de pacientes con diagnostico clinico de tromboastenia de glanzmann, en las cuales hemos hallado cuatro nuevas mutaciones, todas ellas localizadas en la subunidad gpiib. El analisis cuantitativo del rnam de gpiib y gpiiia en plaquetas y el estudio funcional de las mutaciones halladas demuestran que los mecanismos moleculares responsables de la falta de expresion y\/o funcion de complejos gpiib\/iiia han sido: ausencia de rnam, incapacidad de la subunidad mutada para formar heterodimeros, alteracion en la velocidad de transito y\/o ensamblaje en la membrana plasmatica de complejos gpiib\/iiia, alteraci\u00f3n de los mecanismos de se\u00f1alizaci\u00f3n intracelular.  hemos demostrado que la disponibilidad de rnam de gpiib es limitante para la expresi\u00f3n superficial de receptores gpiib\/iiia. Tambien observamos que la disminuci\u00f3n en la tasa de expresi\u00f3n de gpiib\/iiia en mutaciones heterocigotas puede ser debida a un efecto \u00abdominante negativo\u00bb de las formas mutadas de estas proteinas o el resultado de interacciones anomalas con chaperonas del reticulo endoplasmatico.  realizamos estudios de mutagenesis especifica como metodo experimental para establecer una correlacion precisa entre estructura y funci\u00f3n de estas proteinas. Los resultados obtenidos nos permiten afirmar: (a) el puente disulfuro intracateriano 674-687 de gpiib es esencial para mantener una tasa de expresion superficial de gpiib\/iiia normal. (B) la carga negativa del residuo 324 e de gpiib es fundamental para la correcta formacion de heterodimeros con gpiia. (C) los segmentos transmembranar y citoplasmico de gpiiia son imprescindibles para la expresi\u00f3n de gpiib\/iiia. (D) la deleci\u00f3n de los residuos 616-69<\/p>\n<p>&nbsp;<\/p>\n<h3>Datos acad\u00e9micos de la tesis doctoral \u00ab<strong>Fisiopatolog\u00eda del receptor plaquetario de fibrinogeno<\/strong>\u00ab<\/h3>\n<ul>\n<li><strong>T\u00edtulo de la tesis:<\/strong>\u00a0 Fisiopatolog\u00eda del receptor plaquetario de fibrinogeno <\/li>\n<li><strong>Autor:<\/strong>\u00a0 Elena Garcia Arias-salgado <\/li>\n<li><strong>Universidad:<\/strong>\u00a0 Complutense de Madrid<\/li>\n<li><strong>Fecha de lectura de la tesis:<\/strong>\u00a0 24\/05\/2001<\/li>\n<\/ul>\n<p>&nbsp;<\/p>\n<h3>Direcci\u00f3n y tribunal<\/h3>\n<ul>\n<li><strong>Director de la tesis<\/strong>\n<ul>\n<li>Roberto Parrilla Sanchez<\/li>\n<\/ul>\n<\/li>\n<li><strong>Tribunal<\/strong>\n<ul>\n<li>Presidente del tribunal: Juan  Luis Tamargo men\u00e9ndez <\/li>\n<li>consuelo Gonzalez moncloa (vocal)<\/li>\n<li> Jose-carlos garcia-borou Martinez Jos\u00e9 Carlos (vocal)<\/li>\n<li>german Rivas caballero (vocal)<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n<p>&nbsp;<\/p>\n","protected":false},"excerpt":{"rendered":"<p>Tesis doctoral de Elena Garcia Arias-salgado La tromboastenia de glanzmann es una enfermedad hereditaria caracterizada por un defecto cuantitativo o [&hellip;]<\/p>\n","protected":false},"author":1,"featured_media":0,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"site-sidebar-layout":"default","site-content-layout":"","ast-site-content-layout":"","site-content-style":"default","site-sidebar-style":"default","ast-global-header-display":"","ast-banner-title-visibility":"","ast-main-header-display":"","ast-hfb-above-header-display":"","ast-hfb-below-header-display":"","ast-hfb-mobile-header-display":"","site-post-title":"","ast-breadcrumbs-content":"","ast-featured-img":"","footer-sml-layout":"","theme-transparent-header-meta":"","adv-header-id-meta":"","stick-header-meta":"","header-above-stick-meta":"","header-main-stick-meta":"","header-below-stick-meta":"","astra-migrate-meta-layouts":"default","ast-page-background-enabled":"default","ast-page-background-meta":{"desktop":{"background-color":"var(--ast-global-color-4)","background-image":"","background-repeat":"repeat","background-position":"center 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