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

The HD iPSC Consortium, Lim, Ryan G, Salazar, Lisa L, Wilton, Daniel K, King, Alvin R, Stocksdale, Jennifer T, Sharifabad, Delaram, Lau, Alice L +44 more·2017·Nature Neuroscience

Cited by (224)

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  4. Functional analysis of brain derived neurotrophic factor (BDNF) in Huntington’s disease
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  7. Impaired inhibitory GABAergic synaptic transmission and transcription studied in single neurons by Patch-seq in Huntington’s disease
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  8. When repetita no-longer iuvant: somatic instability of the CAG triplet in Huntington’s disease
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  10. Characterization of Neurodevelopmental Abnormalities in iPSC-Derived Striatal Cultures from Patients with Huntington’s Disease
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  11. Directly Reprogrammed Huntington's Disease Neural Precursor Cells Generate Striatal Neurons Exhibiting Aggregates and Impaired Neuronal Maturation
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  12. Enhanced cerebral branched-chain amino acid metabolism in R6/2 mouse model of Huntington’s disease
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  13. Pluripotent stem cell-derived models of neurological diseases reveal early transcriptional heterogeneity
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  14. Juvenile Huntington’s Disease and Other PolyQ Diseases, Update on Neurodevelopmental Character and Comparative Bioinformatic Review of Transcriptomic and Proteomic Data
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  17. Cell Reprogramming to Model Huntington’s Disease: A Comprehensive Review
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  18. Immortalized striatal precursor neurons from Huntington’s disease patient-derived iPS cells as a platform for target identification and screening for experimental therapeutics
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  19. Patient-Specific iPSCs-Based Models of Neurodegenerative Diseases: Focus on Aberrant Calcium Signaling
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  20. Expanded huntingtin CAG repeats disrupt the balance between neural progenitor expansion and differentiation in human cerebral organoids
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  21. Neural Stem Cell Dysfunction in Human Brain Disorders
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  22. Chromatin accessibility and transcription dynamics during in vitro astrocyte differentiation of Huntington’s Disease Monkey pluripotent stem cells
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  24. ISX-9 potentiates CaMKIIδ-mediated BMAL1 activation to enhance circadian amplitude
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  26. Mutant Huntingtin Affects Diabetes and Alzheimer’s Markers in Human and Cell Models of Huntington’s Disease
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  28. An altered extracellular matrix–integrin interface contributes to Huntington’s disease-associated CNS dysfunction in glial and vascular cells
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  30. RUES2 hESCs exhibit MGE-biased neuronal differentiation and muHTT-dependent defective specification hinting at SP1
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  31. RNA-seq analysis reveals significant transcriptome changes in huntingtin-null human neuroblastoma cells
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  32. Altered Activity-Regulated H3K9 Acetylation at TGF-Beta Signaling Genes During Egocentric Memory in Huntington's Disease
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  33. Mutant huntingtin confers cell-autonomous phenotypes on Huntington’s disease iPSC-derived microglia
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  34. Reduced Expression of GABAA Receptor Alpha2 Subunit Is Associated With Disinhibition of DYT-THAP1 Dystonia Patient-Derived Striatal Medium Spiny Neurons
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  35. Haplotype-specific insertion-deletion variations for allele-specific targeting in Huntington's disease
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  36. Human iPS Cell-Derived Patient Tissues and 3D Cell Culture Part 1: Target Identification and Lead Optimization
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  37. Corticostriatal maldevelopment in the R6/2 mouse model of juvenile Huntington's disease
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  38. HTT loss-of-function contributes to RNA deregulation in developing Huntington’s disease neurons
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  39. Induced pluripotent stem cells from Huntington’s disease patients: a promising approach to define and correct disease-related alterations
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  40. Revisiting cell and gene therapies in Huntington’s disease
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  44. Neural stem cells derived from the developing forebrain of YAC128 mice exhibit pathological features of Huntington’s disease
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  52. Elevated SLC7A2 expression is associated with an abnormal neuroinflammatory response and nitrosative stress in Huntington’s disease
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  53. From Neuronal Differentiation of iPSCs to 3D Neural Organoids: Modeling of Neurodegenerative Diseases
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  58. Oligonucleotides Targeting DNA Repeats Downregulate Huntingtin Gene Expression in Huntington's Patient-Derived Neural Model System
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  59. Isoform-Specific Reduction of the Basic Helix-Loop-Helix Transcription Factor TCF4 Levels in Huntington’s Disease
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  71. Cerulenin partially corrects the disrupted developmental transcriptomic signature in Huntington’s disease striatal medium spiny neurons
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