{"id":17543,"date":"2018-03-09T09:05:39","date_gmt":"2018-03-09T09:05:39","guid":{"rendered":"https:\/\/www.deberes.net\/tesis\/sin-categoria\/bases-moleculares-de-las-lipofuscinosis-ceroideas-neuronales\/"},"modified":"2018-03-09T09:05:39","modified_gmt":"2018-03-09T09:05:39","slug":"bases-moleculares-de-las-lipofuscinosis-ceroideas-neuronales","status":"publish","type":"post","link":"https:\/\/www.deberes.net\/tesis\/quimica\/bases-moleculares-de-las-lipofuscinosis-ceroideas-neuronales\/","title":{"rendered":"Bases moleculares de las lipofuscinosis ceroideas neuronales"},"content":{"rendered":"<h2>Tesis doctoral de <strong> Adoraci\u00f3n Villarroya P\u00e9rez <\/strong><\/h2>\n<p>El objetivo general de este trabajo ha sido avanzar en el conocimiento de las bases moleculares de las lipofuscinosis ceroideas neuronales (lcn). las lcn constituyen el grupo de enfermedades neurodegenerativas m\u00e1s frecuentes en ni\u00f1os de pa\u00edses desarrollados. En este estudio concluimos que el mecanismo m\u00e1s probable por el que la subunidad c de la atp sintasa mitocondrial se acumula en los lisosomas de pacientes en la mayor\u00eda de las lcn es la v\u00eda macroautof\u00e1gica. Adem\u00e1s, estudiando la funcionalidad de los sistemas degradativos intracelulares, observamos que las v\u00edas macroautof\u00e1gicas lisosomales est\u00e1n afectadas en las c\u00e9lulas procedentes de pacientes con las formas infantil tard\u00eda y juvenil de las lcn. Sin embargo, otras v\u00edas degradativas lisosomales nomacroatof\u00e1gicas parecen funcionar m\u00e1s eficientemente, mientras que otras v\u00edas de degradaci\u00f3n intracelular de prote\u00ednas (como las que implican a los diferentes proteasomas) no parecen estar alteradas en esta patolog\u00eda.  una segunda parte de nuestro trabajo ha consistido en el estudio de la localizaci\u00f3n y la funci\u00f3n de la prote\u00edna cln3p, implicada en la forma juvenil de las lcn. Por estudios de inmunofluorescencia y subfraccionamiento celular concluimos que cln3p es una prote\u00edna integral de la membrana lisosomal. adem\u00e1s, realizando un estudio comparativo entre c\u00e9lulas procedentes de individuos control y c\u00e9lulas procedentes de la forma juvenil de las lcn con \u00abmicroarrays\u00bb de adn de 1,176 genes, establecimos una expresi\u00f3n elevada de 12 de ellos. De entre todos estos genes, nos parece destacable la elevada expresi\u00f3n del que codifica la subundiad reguladora de las calpa\u00ednas, lo que podr\u00eda indicar que esta familia de ciste\u00edn-proteasas citos\u00f3licas est\u00e1 activada en la enfermedad.  por \u00fatlimo, estudiamos las lcn en poblaci\u00f3n espa\u00f1ola, encontrando que la frecuencia de las principales formas cl\u00ednicas de esta patolog\u00eda es similar a la de poblaciones parecidas a la nue<\/p>\n<p>&nbsp;<\/p>\n<h3>Datos acad\u00e9micos de la tesis doctoral \u00ab<strong>Bases moleculares de las lipofuscinosis ceroideas neuronales<\/strong>\u00ab<\/h3>\n<ul>\n<li><strong>T\u00edtulo de la tesis:<\/strong>\u00a0 Bases moleculares de las lipofuscinosis ceroideas neuronales <\/li>\n<li><strong>Autor:<\/strong>\u00a0 Adoraci\u00f3n Villarroya P\u00e9rez <\/li>\n<li><strong>Universidad:<\/strong>\u00a0 Universitat de val\u00e9ncia (estudi general)<\/li>\n<li><strong>Fecha de lectura de la tesis:<\/strong>\u00a0 18\/06\/2002<\/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 Erwin Knecht<\/li>\n<\/ul>\n<\/li>\n<li><strong>Tribunal<\/strong>\n<ul>\n<li>Presidente del tribunal: salvador Mormeneo bernat <\/li>\n<li>julio Montoya villarroya (vocal)<\/li>\n<li>Fernando Aniento company (vocal)<\/li>\n<li>  (vocal)<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n<p>&nbsp;<\/p>\n","protected":false},"excerpt":{"rendered":"<p>Tesis doctoral de Adoraci\u00f3n Villarroya P\u00e9rez El objetivo general de este trabajo ha sido avanzar en el conocimiento de las [&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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