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Emerging evidence suggests that mutant huntingtin (mHTT) disrupts early neurodevelopment, although the contribution of developmental alterations to the late disease onset remains to be clarified. Leveraging human pluripotent stem cell-derived brain organoids, we and others are exploring how mHTT affects the developing human brain. These models reveal impaired neural progenitor organization and function, accompanied by a mitochondrial stress response, indicating reduced capacity to manage cellular stress. Enhancing mitochondrial health and promoting neural cell resilience may thus represent potential strategies for improving the brain's compensatory mechanisms, thereby prolonging a healthy state. These insights highlight a potential window of opportunity for therapeutic interventions. Targeting mitochondrial fitness and neurodevelopmental pathways at early stages \u2013 long before clinical symptoms emerge \u2013 could help prevent or delay disease onset and progression in affected individuals.<\/jats:p>","DOI":"10.1242\/dmm.052510","type":"journal-article","created":{"date-parts":[[2025,10,28]],"date-time":"2025-10-28T08:14:41Z","timestamp":1761639281000},"source":"Crossref","is-referenced-by-count":3,"title":["Brain organoid models of Huntington's disease shift the focus towards neurodevelopment"],"prefix":"10.1242","volume":"18","author":[{"given":"Wenqing","family":"Xu","sequence":"first","affiliation":[{"name":"Heinrich Heine University 1 Faculty of Mathematics and Natural Sciences , , 40225 D\u00fcsseldorf ,","place":["Germany"]},{"name":"University Hospital D\u00fcsseldorf, Heinrich Heine University 2 Department of General Pediatrics, Neonatology and Pediatric Cardiology, Medical Faculty , , 40225 D\u00fcsseldorf ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9457-1952","authenticated-orcid":false,"given":"Alessandro","family":"Prigione","sequence":"additional","affiliation":[{"name":"University Hospital D\u00fcsseldorf, Heinrich Heine University 2 Department of General Pediatrics, Neonatology and Pediatric Cardiology, Medical Faculty , , 40225 D\u00fcsseldorf ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"237","published-online":{"date-parts":[[2025,10,28]]},"reference":[{"key":"2025102804143877300_DMM052510C1","doi-asserted-by":"publisher","first-page":"2023.04.30.538872","DOI":"10.1101\/2023.04.30.538872","article-title":"Transcriptomic characterization reveals disrupted medium spiny neuron trajectories in huntington's disease and possible therapeutic avenues","author":"Aguirre","year":"2023","journal-title":"bioRxiv"},{"key":"2025102804143877300_DMM052510C2","doi-asserted-by":"publisher","first-page":"28","DOI":"10.1016\/j.cels.2018.05.010","article-title":"High-throughput functional analysis distinguishes pathogenic, nonpathogenic, and compensatory transcriptional changes in neurodegeneration","volume":"7","author":"Al-Ramahi","year":"2018","journal-title":"Cell Syst."},{"key":"2025102804143877300_DMM052510C3","doi-asserted-by":"publisher","first-page":"366","DOI":"10.1016\/0166-2236(89)90074-X","article-title":"The functional anatomy of basal ganglia disorders","volume":"12","author":"Albin","year":"1989","journal-title":"Trends Neurosci."},{"key":"2025102804143877300_DMM052510C4","doi-asserted-by":"publisher","first-page":"253","DOI":"10.1016\/j.stem.2012.04.026","article-title":"Genetic correction of Huntington's disease phenotypes in induced pluripotent stem cells","volume":"11","author":"An","year":"2012","journal-title":"Cell Stem Cell"},{"key":"2025102804143877300_DMM052510C5","doi-asserted-by":"publisher","first-page":"23","DOI":"10.1146\/annurev-neuro-111020-090812","article-title":"Challenges of organoid research","volume":"45","author":"Andrews","year":"2022","journal-title":"Annu. 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