{"id":1125,"date":"2025-10-23T21:16:21","date_gmt":"2025-10-23T19:16:21","guid":{"rendered":"https:\/\/emanuelabottanilab.it\/progetti\/stimolazione-farmacologica-del-metabolismo-mitocondriale-per-promuovere-lo-sviluppo-neuronale-nella-sindrome-di-allan-herndon-dudley\/"},"modified":"2025-11-23T15:46:22","modified_gmt":"2025-11-23T14:46:22","slug":"pharmacological-stimulation-of-mitochondrial-metabolism-to-promote-full-neuronal-development-in-allan-herndon-dudley-syndrome","status":"publish","type":"progetti","link":"https:\/\/emanuelabottanilab.it\/en\/projects\/pharmacological-stimulation-of-mitochondrial-metabolism-to-promote-full-neuronal-development-in-allan-herndon-dudley-syndrome\/","title":{"rendered":"Pharmacological stimulation of mitochondrial metabolism to promote full neuronal development in Allan-Herndon-Dudley Syndrome"},"content":{"rendered":"","protected":false},"template":"","meta":{"_gspb_post_css":""},"class_list":["post-1125","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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T3 deficiency in the brain profoundly disrupts neurogenesis, myelination, and synapse formation, leading to irreversible defects in nervous system development.<\/span><span class=\"NormalTextRun SCXW178455362 BCX0\" data-ccp-parastyle=\"Normal (Web)\">\u00a0 <\/span><span class=\"NormalTextRun SCXW178455362 BCX0\" data-ccp-parastyle=\"Normal (Web)\">T3 plays a crucial role in regulating mitochondrial metabolism, an essential process that sustains the high energy demand of progenitor cells during differentiation. Recent evidence, including that generated by our group, suggests that T3 deficiency impairs mitochondrial function, leading to<\/span><span class=\"NormalTextRun SCXW178455362 BCX0\" data-ccp-parastyle=\"Normal (Web)\">\u00a0reduced neuronal maturation. Conversely, pharmacological stimulation of mitochondrial metabolism can promote neuronal maturation even in the absence of T3, suggesting that targeting this pathway may <\/span><span class=\"NormalTextRun SCXW178455362 BCX0\" data-ccp-parastyle=\"Normal (Web)\">represent<\/span><span class=\"NormalTextRun SCXW178455362 BCX0\" data-ccp-parastyle=\"Normal (Web)\"> a potential therapeutic strategy.<\/span> <span class=\"NormalTextRun SCXW178455362 BCX0\" data-ccp-parastyle=\"Normal (Web)\">Based on these premises, our project aims to gather preclinical evidence on the efficacy of mitochondrial metabolism modulation as an innovative approach for AHDS. Using cellular and mouse models of the disease, we aim to assess the impact of molecules that enhance mitochondrial biogenesis and activity on neuronal maturation, as well as their ability to reach the central nervous system and <\/span><span class=\"NormalTextRun SCXW178455362 BCX0\" data-ccp-parastyle=\"Normal (Web)\">ameliorate<\/span><span class=\"NormalTextRun SCXW178455362 BCX0\" data-ccp-parastyle=\"Normal (Web)\"> pathological phenotypes.<\/span><span class=\"NormalTextRun SCXW178455362 BCX0\" data-ccp-parastyle=\"Normal (Web)\">\u00a0<\/span><\/span><span class=\"EOP SCXW178455362 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":"Nov 2023 \u2013 ongoing","ente_finanziatore_progetto":"Telethon Foundation, Italian Ministry of University and Research","associazione_pazienti_progetto":"<p><a href=\"https:\/\/www.unavitarara.it\/it\/home\/\" target=\"_blank\" rel=\"noopener\"><span class=\"NormalTextRun SCXW200011492 BCX0\" data-ccp-parastyle=\"Normal (Web)\">Una Vita Rara<\/span><span class=\"NormalTextRun SCXW200011492 BCX0\" data-ccp-parastyle=\"Normal (Web)\"> AHDS- MCT<\/span><span class=\"NormalTextRun SCXW200011492 BCX0\" data-ccp-parastyle=\"Normal (Web)\">8 Onlus<\/span><\/a><\/p>\n","linkesterni_progetti":"<p><a href=\"https:\/\/www.fondazionetelethon.it\/cosa-facciamo\/ricerca\/progetti-finanziati\/stimolazione-farmacologica-del-metabolismo-mitocondriale-come-approccio-terapeutico-in-un-modello-murino-di-sindrome-di-allan-herndon-dudley-progetto-in-memoria-di-manuel\/\">Telethon Foundation<\/a><\/p>\n"},"_links":{"self":[{"href":"https:\/\/emanuelabottanilab.it\/en\/wp-json\/wp\/v2\/progetti\/1125","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=1125"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}