{"id":61260,"date":"2007-09-11T00:00:00","date_gmt":"2007-09-11T00:00:00","guid":{"rendered":"https:\/\/www.deberes.net\/tesis\/sin-categoria\/analisis-de-la-funcion-molecular-de-las-protea%c2%adnas-muscleblind-de-drosophila\/"},"modified":"2007-09-11T00:00:00","modified_gmt":"2007-09-11T00:00:00","slug":"analisis-de-la-funcion-molecular-de-las-protea%c2%adnas-muscleblind-de-drosophila","status":"publish","type":"post","link":"https:\/\/www.deberes.net\/tesis\/ciencias-de-la-vida\/analisis-de-la-funcion-molecular-de-las-protea%c2%adnas-muscleblind-de-drosophila\/","title":{"rendered":"\u00aban\u00e1lisis de la funci\u00f3n molecular de las prote\u00ednas muscleblind de drosophila\u00bb"},"content":{"rendered":"

Tesis doctoral de Marta Vicente Crespo <\/strong><\/h2>\n

The human muscleblind-like proteins mbnll-3 bind rnas through pairs of zinc fingers of the cys3his type. They havbthe ability to modify alternative splicing and sub-cellular localisation of defined transcripts and their function is impaired in myotonic dystrophies type 1 and 2 (dml and dm2). Dms are autosomal dominant neuromuscular diseases characterized by myotonia, muscle weakness, and iridescent cataracts, among other symptoms. At a molecular level, dm patients show disruption of alternativf splicing regulation of specific transcripts. The genetic cause of these diseases is the expansioh of either a ctg or a cctg repeat in non-coding regions of the dmp k (dm1) and znf9 (dm2) genes. Upon transcription, expanded rnas form stable hairpins, which sequester nuclear factor~ depleting them from their normal function. Among those factors are the mbnl proteins. The ~elevance of mbnl sequestration in dm pathogenesis is supported by muscleblind like 1 kno6k-out mice (mbnllde3ide3), which reproduce the main features of dm patients including myot6nia, cataracts, and rna splicing defects in transcripts such as troponint 2 and 3. Furthermore,exptession ofmbnll protein reverts the dm-like alterations ofmice expressing expanded cpg containihg rna. the drosophila genomt contains a single muscle blind gene (mb\/), which underg~es alternative splicing giving rise to fjur transcripts coding proteins sharing the amino terminus. Mbla, band c contain two zinc finger kotifs whereas mb1d just one. Mbl function is involved in terminal muscle and photoreceptor diffe~entiation. Mutants carrying strong loss-of- function alleles die as first instar larvae unable to hatchl vltrastructural studies revealed that the regular organization of their sarcomeres was comp~omised. Z-bands were absent mimicking a wild-type muscle in a supercontra:cted state._muscleblind mutant embryos also showed a strong reduction of extra-cellular matrix material at the ijdirect muscle attachments. Apart from these<\/p>\n

 <\/p>\n

Datos acad\u00e9micos de la tesis doctoral \u00ab\u00aban\u00e1lisis de la funci\u00f3n molecular de las prote\u00ednas muscleblind de drosophila\u00bb<\/strong>\u00ab<\/h3>\n
    \n
  • T\u00edtulo de la tesis:<\/strong>\u00a0 \u00aban\u00e1lisis de la funci\u00f3n molecular de las prote\u00ednas muscleblind de drosophila\u00bb <\/li>\n
  • Autor:<\/strong>\u00a0 Marta Vicente Crespo <\/li>\n
  • Universidad:<\/strong>\u00a0 Universitat de val\u00e9ncia (estudi general)<\/li>\n
  • Fecha de lectura de la tesis:<\/strong>\u00a0 09\/11\/2007<\/li>\n<\/ul>\n

     <\/p>\n

    Direcci\u00f3n y tribunal<\/h3>\n
      \n
    • Director de la tesis<\/strong>\n
        \n
      • Rub\u00e9n Artero Allepuz<\/li>\n<\/ul>\n<\/li>\n
      • Tribunal<\/strong>\n
          \n
        • Presidente del tribunal: margarita Cervera jover <\/li>\n
        • Galindo orozco m\u00e1ximo ibo (vocal)<\/li>\n
        • Palacios de castro m.isabel (vocal)<\/li>\n
        • darren Monckton (vocal)<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n

           <\/p>\n","protected":false},"excerpt":{"rendered":"

          Tesis doctoral de Marta Vicente Crespo The human muscleblind-like proteins mbnll-3 bind rnas through pairs of zinc fingers of the […]<\/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":[35,962],"tags":[135329,135327,14958,135326,135328,119954],"class_list":["post-61260","post","type-post","status-publish","format-standard","hentry","category-ciencias-de-la-vida","category-genetica","tag-darren-monckton","tag-galindo-orozco-maximo-ibo","tag-margarita-cervera-jover","tag-marta-vicente-crespo","tag-palacios-de-castro-m-isabel","tag-ruben-artero-allepuz"],"_links":{"self":[{"href":"https:\/\/www.deberes.net\/tesis\/wp-json\/wp\/v2\/posts\/61260","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=61260"}],"version-history":[{"count":0,"href":"https:\/\/www.deberes.net\/tesis\/wp-json\/wp\/v2\/posts\/61260\/revisions"}],"wp:attachment":[{"href":"https:\/\/www.deberes.net\/tesis\/wp-json\/wp\/v2\/media?parent=61260"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/www.deberes.net\/tesis\/wp-json\/wp\/v2\/categories?post=61260"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/www.deberes.net\/tesis\/wp-json\/wp\/v2\/tags?post=61260"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}