{"id":68822,"date":"2018-03-09T23:13:15","date_gmt":"2018-03-09T23:13:15","guid":{"rendered":"https:\/\/www.deberes.net\/tesis\/sin-categoria\/metabolismo-del-hemo-en-un-modelo-murino-de-protoporfiria-eritropoyetica-efecto-de-las-alteraciones-de-la-sa%c2%adntesis-del-hemo-en-la-actividad-de-la-cadena-respiratoria-mitocondrial\/"},"modified":"2018-03-09T23:13:15","modified_gmt":"2018-03-09T23:13:15","slug":"metabolismo-del-hemo-en-un-modelo-murino-de-protoporfiria-eritropoyetica-efecto-de-las-alteraciones-de-la-sa%c2%adntesis-del-hemo-en-la-actividad-de-la-cadena-respiratoria-mitocondrial","status":"publish","type":"post","link":"https:\/\/www.deberes.net\/tesis\/sin-categoria\/metabolismo-del-hemo-en-un-modelo-murino-de-protoporfiria-eritropoyetica-efecto-de-las-alteraciones-de-la-sa%c2%adntesis-del-hemo-en-la-actividad-de-la-cadena-respiratoria-mitocondrial\/","title":{"rendered":"Metabolismo del hemo en un modelo murino de protoporfiria eritropoy\u00e9tica. efecto de las alteraciones de la s\u00edntesis del hemo en la actividad de la cadena respiratoria mitocondrial."},"content":{"rendered":"<h2>Tesis doctoral de <strong> Susana Navarro Ordo\u00f1\u00e9z <\/strong><\/h2>\n<p>La protoporfir\u00eda eritropoy\u00e9tica (ppe),es una enfermedad de herencia autos\u00f3mica dominante caracterizada por la deficiencia de la \u00faltima enzima de la ruta de s\u00edntesis del hemo, la ferroquelatasa (fech).Como consecuencia de este bloqueo enzim\u00e1tico se produce acumulaci\u00f3n de protoporfirina en todos los tejidos (m\u00e9dula \u00f3sea, eritrocitos, plasma, h\u00edgado, piel, etc).La manifestaci\u00f3n m\u00e1s frecuente de esta enfermedad es la fotosensibilada cut\u00e1nea, aunque un 5-10% de los pacientes desarrollan insuficiencia hep\u00e1tica, la cual , puede llegar a requerir transaplante hep\u00e1tico al comprometer la vida del paciente.  el rat\u00f3n homocigoto para la mutaci\u00f3n fech m1pas\/fech m1pas de ppe, constitiye un modelo murino en el que reproduce la enfermedad humana en el grado de mayro severidad:fotosensibilidad cut\u00e1nea y hepatopat\u00eda.  dado que la mayor\u00ed de los componentes de la cadena respiratoria mitocondrial (crm)son hemoprote\u00ednas, resulta l\u00f3gico suponer que las alteraciones del metagbolismo del hemo pueden afectar a las actividades de este sistema de transporte electr\u00f3nico , que podr\u00eda estar relacionado con el desarrollo de la hepatopt\u00eda.Esta tesis estud\u00eda si la hipoactividad de la enzima ferroquelatasa y\/o la acumulaci\u00f3n de protoporfirina en ratones fechm1pas\/fech m1pas puede alterar la s\u00edntesis y\/o actividad de  estas hemoproteinas hep\u00e1ticas.  los resultados obtenidos en este trabajo sugieren que el incremento de la actividad de los complejos de la crm, a trav\u00e9s de una mayor s\u00edntesis de atp,protege frente al efecto hepatot\u00f3xico de la protoporfirina acumulada en el modelo de ppe utilizado.  palabras clave:ferroquelatasa,citocromo ,citcromo c oxidadas.<\/p>\n<p>&nbsp;<\/p>\n<h3>Datos acad\u00e9micos de la tesis doctoral \u00ab<strong>Metabolismo del hemo en un modelo murino de protoporfiria eritropoy\u00e9tica. efecto de las alteraciones de la s\u00edntesis del hemo en la actividad de la cadena respiratoria mitocondrial.<\/strong>\u00ab<\/h3>\n<ul>\n<li><strong>T\u00edtulo de la tesis:<\/strong>\u00a0 Metabolismo del hemo en un modelo murino de protoporfiria eritropoy\u00e9tica. efecto de las alteraciones de la s\u00edntesis del hemo en la actividad de la cadena respiratoria mitocondrial. <\/li>\n<li><strong>Autor:<\/strong>\u00a0 Susana Navarro Ordo\u00f1\u00e9z <\/li>\n<li><strong>Universidad:<\/strong>\u00a0 Complutense de Madrid<\/li>\n<li><strong>Fecha de lectura de la tesis:<\/strong>\u00a0 30\/06\/2004<\/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>Antonio Fontanellas Roma<\/li>\n<\/ul>\n<\/li>\n<li><strong>Tribunal<\/strong>\n<ul>\n<li>Presidente del tribunal: amando Garrido pertierra <\/li>\n<li>Rafael Garesse alarc\u00f3n (vocal)<\/li>\n<li>Juan  Antonio Bueren roncero (vocal)<\/li>\n<li>ricardo More\u00f1o otero (vocal)<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n<p>&nbsp;<\/p>\n","protected":false},"excerpt":{"rendered":"<p>Tesis doctoral de Susana Navarro Ordo\u00f1\u00e9z La protoporfir\u00eda eritropoy\u00e9tica (ppe),es una enfermedad de herencia autos\u00f3mica dominante caracterizada por la deficiencia [&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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