{"id":59529,"date":"2018-03-09T22:47:21","date_gmt":"2018-03-09T22:47:21","guid":{"rendered":"https:\/\/www.deberes.net\/tesis\/sin-categoria\/genomica-funcional-del-sistema-oxphos\/"},"modified":"2018-03-09T22:47:21","modified_gmt":"2018-03-09T22:47:21","slug":"genomica-funcional-del-sistema-oxphos","status":"publish","type":"post","link":"https:\/\/www.deberes.net\/tesis\/bioquimica-molecular\/genomica-funcional-del-sistema-oxphos\/","title":{"rendered":"Genomica funcional del sistema oxphos"},"content":{"rendered":"<h2>Tesis doctoral de <strong> Gustavo Ferrin S\u00e1nchez <\/strong><\/h2>\n<p>La cadena respiratoria mitocondrial est\u00e1 compuesta por, aproximadamente, 100 prote\u00ednas diferentes. De estas, solamente 13 seencuentran codificadas en el mtdna; el resto est\u00e1n codificadas en genes nucleares. De hecho, se cree que varios cientos de genesnucleares son necesarios para las funciones del sistema de fosforilaci\u00f3n oxidativa (oxphos). As\u00ed, las prote\u00ednas mitocondrialesrepresentan m\u00e1s del 3 % del total de prote\u00ednas celulares.Hoy d\u00eda, se asume que la mitocondria contiene m\u00e1s de 1000 prote\u00ednas diferentes (scharfe et al., 2000), cuyos genes son posiblescandidatos de des\u00f3rdenes mitocondriales. Los defectos que se producen en el mtdna probablemente explican la mitad de losdesordenes mit\u00f3condriales del sistema oxphos. Sin embargo, la causa del 50 % de los casos restantes no ha sido determinada a\u00fan.Las mutaciones de estos genes nucleares, relacionados directa o indirectamente con el sistema de fosforilaci\u00f3n oxidativa, podr\u00edanoriginar un sistema oxphos anormal (rotig and munnich, 2003).La mitad de los pacientes humanos que presentan una actividad de complejo 1 deficiente debido a problemas en el ensamblaje delmismo, no muestran alteraciones en los componentes estructurales de dicho complejo (janssen et al., 2006). Sin embargo, mientrasque se han descrito diferentes chaperonas necesarias para la formaci\u00f3n de los otros complejos del sistema oxphos, laidentificaci\u00f3n de las prote\u00ednas necesarias para el correcto ensamblaje del complejo 1 de mam\u00edferos ha resultado muy costosa,considerando que se trata del complejo de mayor tama\u00f1o (compuesto por 46 subunidades), con un peso molecular aproximado de 1mda.Como consecuencia de la imposibilidad de manipular directamente el mtdna de mam\u00edferos, y debido a las caracter\u00edsticas tanparticulares de dicho genoma (del que existen miles de copias en cada c\u00e9lula, donde, adem\u00e1s, pueden coexistir mtdna sano ymtdna mutante (heteroplasmia), existiendo un umbral de mutaci\u00f3n que debe ser superado para que se manifieste c<\/p>\n<p>&nbsp;<\/p>\n<h3>Datos acad\u00e9micos de la tesis doctoral \u00ab<strong>Genomica funcional del sistema oxphos<\/strong>\u00ab<\/h3>\n<ul>\n<li><strong>T\u00edtulo de la tesis:<\/strong>\u00a0 Genomica funcional del sistema oxphos <\/li>\n<li><strong>Autor:<\/strong>\u00a0 Gustavo Ferrin S\u00e1nchez <\/li>\n<li><strong>Universidad:<\/strong>\u00a0 Zaragoza<\/li>\n<li><strong>Fecha de lectura de la tesis:<\/strong>\u00a0 29\/06\/2007<\/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>Acisclo P\u00e9rez Martos<\/li>\n<\/ul>\n<\/li>\n<li><strong>Tribunal<\/strong>\n<ul>\n<li>Presidente del tribunal: Manuel Lopez Perez <\/li>\n<li>Rafael Garasse Alarc\u00f3n (vocal)<\/li>\n<li> Osta Pinzolas Mar\u00eda  Rosario (vocal)<\/li>\n<li> Piruat Palomo Jos\u00e9 Ignacio (vocal)<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n<p>&nbsp;<\/p>\n","protected":false},"excerpt":{"rendered":"<p>Tesis doctoral de Gustavo Ferrin S\u00e1nchez La cadena respiratoria mitocondrial est\u00e1 compuesta por, aproximadamente, 100 prote\u00ednas diferentes. De estas, solamente [&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 center","background-size":"auto","background-attachment":"scroll","background-type":"","background-media":"","overlay-type":"","overlay-color":"","overlay-gradient":""},"tablet":{"background-color":"","background-image":"","background-repeat":"repeat","background-position":"center center","background-size":"auto","background-attachment":"scroll","background-type":"","background-media":"","overlay-type":"","overlay-color":"","overlay-gradient":""},"mobile":{"background-color":"","background-image":"","background-repeat":"repeat","background-position":"center center","background-size":"auto","background-attachment":"scroll","background-type":"","background-media":"","overlay-type":"","overlay-color":"","overlay-gradient":""}},"ast-content-background-meta":{"desktop":{"background-color":"var(--ast-global-color-5)","background-image":"","background-repeat":"repeat","background-position":"center center","background-size":"auto","background-attachment":"scroll","background-type":"","background-media":"","overlay-type":"","overlay-color":"","overlay-gradient":""},"tablet":{"background-color":"var(--ast-global-color-5)","background-image":"","background-repeat":"repeat","background-position":"center center","background-size":"auto","background-attachment":"scroll","background-type":"","background-media":"","overlay-type":"","overlay-color":"","overlay-gradient":""},"mobile":{"background-color":"var(--ast-global-color-5)","background-image":"","background-repeat":"repeat","background-position":"center center","background-size":"auto","background-attachment":"scroll","background-type":"","background-media":"","overlay-type":"","overlay-color":"","overlay-gradient":""}},"footnotes":""},"categories":[115,940,13610],"tags":[28642,131524,6284,131526,131527,131525],"class_list":["post-59529","post","type-post","status-publish","format-standard","hentry","category-bioquimica-molecular","category-genetica-bioquimica","category-zaragoza","tag-acisclo-perez-martos","tag-gustavo-ferrin-sanchez","tag-manuel-lopez-perez","tag-osta-pinzolas-maria-rosario","tag-piruat-palomo-jose-ignacio","tag-rafael-garasse-alarcon"],"_links":{"self":[{"href":"https:\/\/www.deberes.net\/tesis\/wp-json\/wp\/v2\/posts\/59529","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/www.deberes.net\/tesis\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/www.deberes.net\/tesis\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/www.deberes.net\/tesis\/wp-json\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/www.deberes.net\/tesis\/wp-json\/wp\/v2\/comments?post=59529"}],"version-history":[{"count":0,"href":"https:\/\/www.deberes.net\/tesis\/wp-json\/wp\/v2\/posts\/59529\/revisions"}],"wp:attachment":[{"href":"https:\/\/www.deberes.net\/tesis\/wp-json\/wp\/v2\/media?parent=59529"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/www.deberes.net\/tesis\/wp-json\/wp\/v2\/categories?post=59529"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/www.deberes.net\/tesis\/wp-json\/wp\/v2\/tags?post=59529"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}