{"id":1051,"date":"2025-10-23T21:25:40","date_gmt":"2025-10-23T19:25:40","guid":{"rendered":"https:\/\/emanuelabottanilab.it\/progetti\/eng-studio-del-ruolo-della-disfunzione-mitocondriale-nella-neurogenesi-e-nelle-malattie-genetiche-rare-del-neurosviluppo\/"},"modified":"2025-11-18T20:47:08","modified_gmt":"2025-11-18T19:47:08","slug":"dissecting-the-role-of-mitochondrial-dysfunction-in-neurogenesis-and-in-rare-neurodevelopmental-diseases","status":"publish","type":"progetti","link":"https:\/\/emanuelabottanilab.it\/en\/projects\/dissecting-the-role-of-mitochondrial-dysfunction-in-neurogenesis-and-in-rare-neurodevelopmental-diseases\/","title":{"rendered":"Dissecting the role of mitochondrial (dys)function in neurogenesis and in rare neurodevelopmental diseases"},"content":{"rendered":"","protected":false},"template":"","meta":{"_gspb_post_css":""},"class_list":["post-1051","progetti","type-progetti","status-publish","hentry"],"blocksy_meta":[],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v27.6 - 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While traditionally classified as early-onset neurodegenerative conditions, recent evidence suggests that primary mitochondrial dysfunction may impair neurogenesis, thereby preventing proper neuronal maturation from<\/span><span class=\"NormalTextRun SCXW31839892 BCX0\" data-ccp-parastyle=\"Normal (Web)\"> the earliest stages of development. In cellular and organoid models, mitochondrial alterations hinder the activation of transcriptional programs essential for neuronal morphogenesis, leading to<\/span><span class=\"NormalTextRun SCXW31839892 BCX0\" data-ccp-parastyle=\"Normal (Web)\">\u00a0the persistence of immature and disorganized phenotypes. These findings suggest that <\/span><span class=\"NormalTextRun SpellingErrorV2Themed SCXW31839892 BCX0\" data-ccp-parastyle=\"Normal (Web)\">pediatric<\/span><span class=\"NormalTextRun SCXW31839892 BCX0\" data-ccp-parastyle=\"Normal (Web)\"> mitochondrial syndromes can be regarded not only as neurodegenerative disorders but also as disorders of neurogenesis.<\/span> <span class=\"NormalTextRun SCXW31839892 BCX0\" data-ccp-parastyle=\"Normal (Web)\">The aim of this project is to elucidate the mechanisms by which mitochondrial dysfunction affects neural development. The working hypothesis is that <\/span><span class=\"NormalTextRun SCXW31839892 BCX0\" data-ccp-parastyle=\"Normal (Web)\">different aspects<\/span><span class=\"NormalTextRun SCXW31839892 BCX0\" data-ccp-parastyle=\"Normal (Web)\"> of metabolic and bioenergetic impairment<\/span><span class=\"NormalTextRun SCXW31839892 BCX0\" data-ccp-parastyle=\"Normal (Web)\">\u00a0represent critical bottlenecks for neuronal generation and maturation. To address this, we will employ integrated approaches in cellular and organoid systems, as well as diverse genetic animal models of mitochondrial dysfunction, including the pig, which provides a particularly relevant paradigm for studying neurodevelopmental defects. The <\/span><span class=\"NormalTextRun AdvancedProofingIssueV2Themed SCXW31839892 BCX0\" data-ccp-parastyle=\"Normal (Web)\">ultimate goal<\/span><span class=\"NormalTextRun SCXW31839892 BCX0\" data-ccp-parastyle=\"Normal (Web)\"> is to elucidate how mitochondrial function shapes neurogenesis and to <\/span><span class=\"NormalTextRun SCXW31839892 BCX0\" data-ccp-parastyle=\"Normal (Web)\">establish<\/span><span class=\"NormalTextRun SCXW31839892 BCX0\" data-ccp-parastyle=\"Normal (Web)\"> its role in the pathogenesis of rare neurodevelopmental disorders.<\/span><\/span><span class=\"EOP SCXW31839892 BCX0\" data-ccp-props=\"{&quot;134233117&quot;:true,&quot;134233118&quot;:true,&quot;201341983&quot;:0,&quot;335551550&quot;:6,&quot;335551620&quot;:6,&quot;335559740&quot;:240,&quot;335572079&quot;:12,&quot;335572080&quot;:1,&quot;335572081&quot;:4278190080,&quot;469789806&quot;:&quot;single&quot;}\">\u00a0<\/span><\/p>\n","coordinatore_progetto":"Emanuela Bottani","durata_progetto":"Oct 2025 - ongoing","ente_finanziatore_progetto":"Italian Ministry of University and Research","associazione_pazienti_progetto":"","linkesterni_progetti":"<p><a href=\"https:\/\/www.univrmagazine.it\/2025\/02\/21\/il-ruolo-chiave-dei-mitocondri-nel-neurosviluppo-e-nella-cura-di-malattie-genetiche-rare\/\" target=\"_blank\" rel=\"noopener\">External link<\/a><\/p>\n"},"_links":{"self":[{"href":"https:\/\/emanuelabottanilab.it\/en\/wp-json\/wp\/v2\/progetti\/1051","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/emanuelabottanilab.it\/en\/wp-json\/wp\/v2\/progetti"}],"about":[{"href":"https:\/\/emanuelabottanilab.it\/en\/wp-json\/wp\/v2\/types\/progetti"}],"wp:attachment":[{"href":"https:\/\/emanuelabottanilab.it\/en\/wp-json\/wp\/v2\/media?parent=1051"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}