{"id":1186,"date":"2020-07-01T14:36:21","date_gmt":"2020-07-01T13:36:21","guid":{"rendered":"http:\/\/www-b3.ijs.si\/?page_id=1186"},"modified":"2020-07-01T14:36:21","modified_gmt":"2020-07-01T13:36:21","slug":"neurodegeneration","status":"publish","type":"page","link":"https:\/\/www-b3.ijs.si\/index.php\/raziskave\/podrocja-raziskav\/neurodegeneration\/?lang=sl","title":{"rendered":"Nevrodegeneracija"},"content":{"rendered":"<p><section class=\"kc-elm kc-css-84487 kc_row\"><div class=\"kc-row-container  kc-container\"><div class=\"kc-wrap-columns\"><div class=\"kc-elm kc-css-398712 kc_col-sm-12 kc_column kc_col-sm-12\"><div class=\"kc-col-container\">\n<div class=\"kc-elm kc-css-950659 kc-blog-posts kc-blog-posts-3 kc-blog-grid kc_blog_masonry kc-image-align-both\">\n\t\n\t\t<h3>Blog Post: Nothing not found.<\/h3>\n\n\t\n<\/div>\n<\/div><\/div><\/div><\/div><\/section><section class=\"kc-elm kc-css-312943 kc_row\"><div class=\"kc-row-container  kc-container\"><div class=\"kc-wrap-columns\"><div class=\"kc-elm kc-css-784921 kc_col-sm-12 kc_column kc_col-sm-12\"><div class=\"kc-col-container\"><div class=\"kc-elm kc-css-468598 kc_text_block\"><\/p>\n<h2><strong>Molekularni mehanizmi nevrodegeneracije<\/strong><\/h2>\n<p>V na\u0161i skupini raziskujemo molekularne procese, povezane z nevrodegenerativnimi obolenji, kot so amiotrofi\u010dna lateralna skleroza (ALS), fronto-temporalna demenca (FTD) in Alzheimerjeva bolezen. Osredoto\u010damo se predvsem na povezavo teh obolenj z okvarami znotrajceli\u010dnega jedrnega transporta, odzivom celic na stres in s spremembami do katerih prihaja pri znotrajceli\u010dnih makromolekularnih interakcijah (interakciji protein-protein in protein-RNA).<br \/>Proteina TDP-43 in FUS spadata med glavne dejavnike povzro\u010ditve agregacije znotrajceli\u010dnih proteinov pri ALS in FTD. To sta prete\u017eno jedrna proteina, ki sodelujeta pri prepisovanju in transportu RNA. Zato je ve\u010dina na\u0161ega dela osredoto\u010denega na jedrni transport teh proteinov, njune vezavne lastnosti za RNA in na patolo\u0161ki pomen njunih interakcij z RNA in ostalimi proteini. Na\u0161e delo sega tudi na podro\u010dje funkcionalne genetike, patologije in molekularnih mehanizmov ekspanzijske mutacije heksanukleotidnih zaporedij v intronu gena C9Orf72. Pokazali smo, da lahko toksi\u010dnost te mutacije izvira iz preobilja zaporedij ponavljajo\u010de se RNA v celici, kot tudi iz preobilja proteinov sestavljenih iz di-peptidnih ponovitev, ki se prevedejo iz tak\u0161ne RNA. Ob tem nam je uspelo dokazati, da RNA iz kodirajo\u010de verige DNA lahko tvori G-kvadruplekse, medtem ko prepisano proti-smiselno zaporedje RNA lahko tvori i-motive \/ protonirane lasnice. Okarakterizirati nam je uspelo proteine, ki ve\u017eejo oba tipa RNA, ki se prepisuje iz C9Orf72..<br \/>Sodelujemo tudi v \u0161tevilnih mednarodnih konzorcijih za <a href=\"https:\/\/www.projectmine.com\/\">genetsko karakterizacijo ALS<\/a>.<\/p>\n<p>\n<\/div><div class=\"kc-elm kc-css-405566 kc_shortcode kc_single_image\">\n\n        <img src=\"https:\/\/www-b3.ijs.si\/wp-content\/uploads\/2020\/11\/Spletna-stran-slovensko-002.png\" class=\"\" alt=\"\" \/>    <\/div>\n<\/div><\/div><\/div><\/div><\/section><section class=\"kc-elm kc-css-208001 kc_row\"><div class=\"kc-row-container  kc-container\"><div class=\"kc-wrap-columns\"><div class=\"kc-elm kc-css-855972 kc_col-sm-12 kc_column kc_col-sm-12\"><div class=\"kc-col-container\"><div class=\"kc-elm kc-css-553887 kc_text_block\"><\/p>\n<h4>Izbrane publikacije<\/h4>\n<ul>\n<li>MALNAR, Mirjana,\u00a0\u010cER\u010cEK, Ur\u0161a, YIN, Xiaoke, TIN HO, Manh, REPI\u010c-LAMPRET, Barbka, NEUMANN, Manuela, HERMANN, Andreas, ROULEAU, Guy, SUTER, Beat, MAYR, Manuel,\u00a0ROGELJ, Boris.\u00a0<strong><a href=\"https:\/\/www.nature.com\/articles\/s41467-023-41511-3\">Phenylalanine-tRNA aminoacylation is compromised by ALS\/FTD-associated C9orf72 C4G2 repeat RNA<\/a><\/strong>.\u00a0Nature communications, 202314, 5764, 1-15<\/li>\n<li>MOTALN, Helena,\u00a0\u010cER\u010cEK, Ur\u0161a, YAMOAH, Alfred, TRIPATHI, Priyanka, ARONICA, Eleonora, GOSWAMI, Anand,\u00a0ROGELJ, Boris.\u00a0<strong><a href=\"https:\/\/academic.oup.com\/brain\/article\/146\/10\/4088\/7127731\">Abl kinase-mediated FUS Tyr526 phosphorylation alters nucleocytoplasmic FUS localization in FTLD-FUS.<\/a><\/strong>\u00a0Brain : journal of neurology, 2023, 37<\/li>\n<li>BO\u017dI\u010c, Janja, MOTALN, Helena, PUCER JANE\u017d, Anja, MARKI\u010c, Lara, ROGELJ, Boris, et al.\u00a0<a href=\"https:\/\/academic.oup.com\/brain\/article\/145\/2\/684\/6372042?login=true\"><strong>Interactome screening of C9orf72 dipeptide repeats reveals VCP sequestration and functional impairment by polyGA.<\/strong><\/a>\u00a0<em>Brain : journal of neurology<\/em>, 2022,145, 2, 684-699.<\/li>\n<li>MALNAR, Mirjana, ROGELJ, Boris.\u00a0<a href=\"https:\/\/jcs.biologists.org\/content\/134\/4\/jcs256602\" target=\"_blank\" rel=\"noopener noreferrer\"><strong>SFPQ regulates the accumulation of RNA foci and dipeptide repeat proteins from the expanded repeat mutation in C9orf72.<\/strong><\/a>\u00a0Journal of cell science, 2021, 28.<\/li>\n<li>MOTALN, Helena, \u010cER\u010cEK, Ur\u0161a, RECEK, Nina, BAJC \u010cESNIK, Ana, MOZETI\u010c, Miran, ROGELJ, Boris.<a href=\"https:\/\/pubs.rsc.org\/en\/content\/articlelanding\/2020\/BM\/D0BM00488J#!divAbstract\" target=\"_blank\" rel=\"noopener noreferrer\"><strong>\u00a0Cold atmospheric plasma induces stress granule formation via an eIF2[alpha]-dependent pathway.<\/strong><\/a>\u00a0Biomaterials science, 2020. 19.<\/li>\n<li>BAJC \u010cESNIK, Ana, DAROVIC, Simona, PRPAR MIHEVC, Sonja, \u0160TALEKAR, Maja, MALNAR, Mirjana, MOTALN, Helena, LEE, Youn-Bok, MAZEJ, Julija, POHLEVEN, Jure, GROSCH, Markus, MODIC, Miha, FONOVI\u0106, Marko, TURK, Boris, DRUKKER, Micha, SHAW, Christopher E., ROGELJ, Boris. <a href=\"https:\/\/jcs.biologists.org\/content\/132\/5\/jcs224303\"><strong>Nuclear RNA foci from C9ORF72 expansion mutation form paraspeckle-like bodies.<\/strong><\/a> Journal of cell science, 2019, 132, 5, 1-14.<\/li>\n<\/ul>\n<p>\n<\/div><div class=\"kc-elm kc-css-536992\" style=\"height: 30px; clear: both; width:100%;\"><\/div><\/div><\/div><\/div><\/div><\/section><section class=\"kc-elm kc-css-611813 kc_row\"><div class=\"kc-row-container  kc-container\"><div class=\"kc-wrap-columns\"><div class=\"kc-elm kc-css-529330 kc_col-sm-12 kc_column kc_col-sm-12\"><div class=\"kc-col-container\"><div class=\"kc-elm kc-css-846004\" style=\"height: 30px; clear: both; width:100%;\"><\/div><\/div><\/div><\/div><\/div><\/section><\/p>\n","protected":false},"excerpt":{"rendered":"","protected":false},"author":6,"featured_media":665,"parent":1183,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","meta":[],"_links":{"self":[{"href":"https:\/\/www-b3.ijs.si\/index.php\/wp-json\/wp\/v2\/pages\/1186"}],"collection":[{"href":"https:\/\/www-b3.ijs.si\/index.php\/wp-json\/wp\/v2\/pages"}],"about":[{"href":"https:\/\/www-b3.ijs.si\/index.php\/wp-json\/wp\/v2\/types\/page"}],"author":[{"embeddable":true,"href":"https:\/\/www-b3.ijs.si\/index.php\/wp-json\/wp\/v2\/users\/6"}],"replies":[{"embeddable":true,"href":"https:\/\/www-b3.ijs.si\/index.php\/wp-json\/wp\/v2\/comments?post=1186"}],"version-history":[{"count":1,"href":"https:\/\/www-b3.ijs.si\/index.php\/wp-json\/wp\/v2\/pages\/1186\/revisions"}],"predecessor-version":[{"id":1891,"href":"https:\/\/www-b3.ijs.si\/index.php\/wp-json\/wp\/v2\/pages\/1186\/revisions\/1891"}],"up":[{"embeddable":true,"href":"https:\/\/www-b3.ijs.si\/index.php\/wp-json\/wp\/v2\/pages\/1183"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/www-b3.ijs.si\/index.php\/wp-json\/wp\/v2\/media\/665"}],"wp:attachment":[{"href":"https:\/\/www-b3.ijs.si\/index.php\/wp-json\/wp\/v2\/media?parent=1186"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}