Self-organizing neuruloids model developmental aspects of Huntington's disease in the ectodermal compartment

T Haremaki, JJ Metzger, T Rito, MZ Ozair, F Etoc… - Nature …, 2019 - nature.com
Harnessing the potential of human embryonic stem cells to mimic normal and aberrant
development with standardized models is a pressing challenge. Here we use micropattern …

Developmental alterations in Huntington's disease neural cells and pharmacological rescue in cells and mice

Nature neuroscience, 2017 - nature.com
Neural cultures derived from Huntington's disease (HD) patient-derived induced pluripotent
stem cells were used for'omics' analyses to identify mechanisms underlying …

Faulty neuronal determination and cell polarization are reverted by modulating HD early phenotypes

P Conforti, D Besusso, VD Bocchi… - Proceedings of the …, 2018 - National Acad Sciences
Increasing evidence suggests that early neurodevelopmental defects in Huntington's
disease (HD) patients could contribute to the later adult neurodegenerative phenotype …

Modeling human telencephalic development and autism-associated SHANK3 deficiency using organoids generated from single neural rosettes

Y Wang, S Chiola, G Yang, C Russell… - Nature …, 2022 - nature.com
Human telencephalon is an evolutionarily advanced brain structure associated with many
uniquely human behaviors and disorders. However, cell lineages and molecular pathways …

Enhanced cortical neural stem cell identity through short SMAD and WNT inhibition in human cerebral organoids facilitates emergence of outer radial glial cells

D Rosebrock, S Arora, N Mutukula, R Volkman… - Nature cell …, 2022 - nature.com
Cerebral organoids exhibit broad regional heterogeneity accompanied by limited cortical
cellular diversity despite the tremendous upsurge in derivation methods, suggesting …

Modeling Huntington's disease with induced pluripotent stem cells

JA Kaye, S Finkbeiner - Molecular and Cellular Neuroscience, 2013 - Elsevier
Huntington's disease (HD) causes severe motor dysfunction, behavioral abnormalities,
cognitive impairment and death. Investigations into its molecular pathology have primarily …

Human Huntington's disease iPSC-derived cortical neurons display altered transcriptomics, morphology, and maturation

SR Mehta, CM Tom, Y Wang, C Bresee, D Rushton… - Cell reports, 2018 - cell.com
Huntington's disease (HD) is a neurodegenerative disease caused by an expanded CAG
repeat in the Huntingtin (HTT) gene. Induced pluripotent stem cell (iPSC) models of HD …

Exploring the regulation of human neural precursor cell differentiation using arrays of signaling microenvironments

Y Soen, A Mori, TD Palmer, PO Brown - Molecular systems biology, 2006 - embopress.org
Cells of a developing embryo integrate a complex array of local and long‐range signals that
act in concert with cell‐intrinsic determinants to influence developmental decisions. To …

A patterned human neural tube model using microfluidic gradients

X Xue, YS Kim, AI Ponce-Arias, R O'Laughlin, RZ Yan… - Nature, 2024 - nature.com
The human nervous system is a highly complex but organized organ. The foundation of its
complexity and organization is laid down during regional patterning of the neural tube, the …

A novel human embryonic stem cell‐derived Huntington's disease neuronal model exhibits mutant huntingtin (mHTT) aggregates and soluble mHTT‐dependent …

B Lu, J Palacino - The FASEB Journal, 2013 - Wiley Online Library
Most neurodegenerative diseases are linked to aberrant accumulation of aggregation‐prone
proteins. Among them, Huntington's disease (HD) is caused by an expanded polyglutamine …