Poster preview
ePoster
AIR-LIQUID INTERFACE CORTICAL ORGANOIDS AS A PLATFORM TO STUDY TDP-43 PATHOLOGY IN ALS
Roberto Montoro Ferrerand 5 co-authors
Department of Translational Neuroscience, UMC Utrecht Brain Center
FENS Forum 2026 (2026)
Barcelona, Spain
Board PS04-08PM-153
Presentation
Date TBA
Board: PS04-08PM-153
Event Information
Poster Board
PS04-08PM-153
Poster
View posterAbstract
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by progressive degeneration of upper and lower motor neurons, leading to muscle weakness, and, in up to 50% of patients, cognitive impairment along the ALS-frontotemporal dementia spectrum. Although ALS is a complex genetic disorder, approximately 97% of patients exhibit TAR DNA-binding protein 43 (TDP-43) proteinopathy, characterized by its nuclear depletion and cytoplasmic aggregation, suggesting a central role for TDP-43 in disease pathogenesis. However, human models that recapitulate TDP-43 pathology remain limited. We therefore aimed to establish and molecularly characterize human induced pluripotent stem cell-derived air-liquid interface cortical organoids (ALI-CO) and to evaluate their suitability for modeling TDP-43 pathology. To comprehensively define cell populations in this model in time, we performed single-cell RNA sequencing across ALI-CO differentiation. To model disease, we aim to exogenously induce TDP-43 pathology and monitor protein mislocalization and aggregation. This approach enables the investigation of neuronal susceptibility and the contribution of specific cell types, such as microglia, to disease pathogenesis.
Recommended posters
MODELING TDP43 PROTEINOPATHY IN ALS USING PATIENT-DERIVED CORTICO-SPINAL ORGANOIDS
Erika Poggianti, Marilyn M. A. Salavarria, Susanna Basteri, Maria Teresa Dell'Anno, Alessio Zanelli, Gianmarco Ferri, Linda Ottoboni, Luciano Conti, Stefania Corti, Marco Onorati
INVESTIGATING TRIPARTITE SYNAPSE PATHOLOGY IN ALS UTILIZING A HIPSC-DERIVED ORGANOID MODEL
Ahmad Jibai, Matthew Broadhead, Neela Murti, Laura Kibedi Makfalvi Varga, Channa Jakobs, Astrid Van der Geest, Kathryn Bowles, Jeroen Pasterkamp, Gareth Miles
HUMAN CORTICAL AND SPINAL CORD ORGANOIDS REVEAL CELL-TYPE–SPECIFIC CONSEQUENCES OF TDP-43 I383V MUTATION
Julia Sala Jarque, Sarah Eger, Ilana Krelstein, Yana Stepanyan, Andrew Longhini, Ana Barbara Cupajita, Pol Andres Benito, Mohamed El-Badry, Jorge Padro Esteve, Kenneth Kosik
A MULTIMODULAR APPROACH TO INVESTIGATE EARLY NEURODEGENERATIVE CHANGES AND POTENTIAL THERAPIES IN TDP-43 PROTEINOPATHIES
Martha Davies-Branch, Daipayan Chowdhury, Mark Baker, Gavin Clowry, Fiona LeBeau
INVESTIGATING NEUROINFLAMMATION IN TRAUMATIC INJURY USING HUMAN STEM-CELL DERIVED ORGANOID WITH AMYOTROPHIC LATERAL SCLEROSIS GENE MUTATION
Teea Wang, Ping Yip, Agnes Nishimura
IDENTIFICATION OF NEURONAL SUBTYPE-SPECIFIC SPLICE VARIANTS IN IPSC-DERIVED CELL MODELS OF ALS AND FTD
Luke Foulser, Aurora Veteleanu, Gianmarco Mastrogiovanni, Hannah Garnett, Magdalena Gamperl, Sejla Salic-Hainzl, Tilmann Buerckstuemmer, Tom Harris-Brown, Amanda Turner, Ben Newman, Karl Firth, Oliver Dovey, Will Bernard, Tony Oosterveen