eprintid: 10204211 rev_number: 6 eprint_status: archive userid: 699 dir: disk0/10/20/42/11 datestamp: 2025-02-03 12:07:48 lastmod: 2025-02-03 12:07:48 status_changed: 2025-02-03 12:07:48 type: article metadata_visibility: show sword_depositor: 699 creators_name: Thirumalai, Srishruthi creators_name: Livesey, Frederick J creators_name: Patani, Rickie creators_name: Hung, Christy title: APP antisense oligonucleotides are effective in rescuing mitochondrial phenotypes in human iPSC-derived trisomy 21 astrocytes ispublished: pub divisions: UCL divisions: B02 divisions: D13 divisions: G22 keywords: Alzheimer's disease, antisense oligonucleotides, APP, astrocytes, Down syndrome, mitochondrial function note: © 2025 The Author(s). Alzheimer's & Dementia published by Wiley Periodicals LLC on behalf of Alzheimer's Association. This is an open access article under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/). abstract: INTRODUCTION: Antisense oligonucleotides (ASOs) have shown promise in reducing amyloid precursor protein (APP) levels in neurons, but their effects in astrocytes, key contributors to neurodegenerative diseases, remain unclear. This study evaluates the efficacy of APP ASOs in astrocytes derived from an individual with Down syndrome (DS), a population at high risk for Alzheimer's disease (AD). METHODS: Human induced pluripotent stem cells (hiPSCs) from a healthy individual and an individual with DS were differentiated into astrocytes. Astrocytes were treated with APP ASOs for 10 days, and APP levels were quantified. Mitochondrial morphology and superoxide production in DS astrocytes were analyzed using super-resolution and confocal microscopy. RESULTS: APP ASOs significantly reduced APP levels in astrocytes from both control and DS individuals. In DS astrocytes, treatment restored mitochondrial health, increasing mitochondrial number and size while reducing superoxide production. DISCUSSION: APP ASOs effectively reduce APP levels and improve mitochondrial health in astrocytes, suggesting their potential as a therapeutic approach for DS and DS-related AD. Further in vivo studies are required to confirm these findings. HIGHLIGHTS: APP ASOs reduce APP levels in human iPSC-derived astrocytes. APP ASO treatment rescues mitochondrial phenotypes in trisomy 21 astrocytes. This study supports ASOs as a potential therapy for Down syndrome-related Alzheimer's disease. date: 2025-01 date_type: published publisher: Wiley official_url: https://doi.org/10.1002/alz.14560 oa_status: green full_text_type: pub language: eng primo: open primo_central: open_green verified: verified_manual elements_id: 2357111 doi: 10.1002/alz.14560 medium: Print lyricists_name: Hung, Oi Ying lyricists_id: OYHHU52 actors_name: Hung, Oi Ying actors_id: OYHHU52 actors_role: owner funding_acknowledgements: [Alzheimer's Research UK] full_text_status: public publication: Alzheimer's & Dementia volume: 21 number: 1 article_number: e14560 event_location: United States citation: Thirumalai, Srishruthi; Livesey, Frederick J; Patani, Rickie; Hung, Christy; (2025) APP antisense oligonucleotides are effective in rescuing mitochondrial phenotypes in human iPSC-derived trisomy 21 astrocytes. Alzheimer's & Dementia , 21 (1) , Article e14560. 10.1002/alz.14560 <https://doi.org/10.1002/alz.14560>. Green open access document_url: https://discovery.ucl.ac.uk/id/eprint/10204211/1/Alzheimer%20s%20%20%20Dementia%20-%202025%20-%20Thirumalai%20-%20APP%20antisense%20oligonucleotides%20are%20effective%20in%20rescuing%20mitochondrial.pdf