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Generation of human induced pluripotent stem cell-derived 3D human cortical, striatal, mesencephalic and diencephalic organoids

ABSTRACT: Generation of human induced pluripotent stem cell-derived 3D human cortical, striatal, mesencephalic and diencephalic organoids

PROVIDER: PRJNA1171745 | ENA |

REPOSITORIES: ENA

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Generation of human induced pluripotent stem cell-derived 3D human cortical, striatal, mesencephalic and diencephalic organoids

Project description:Single cell RNA-seq characterization of stem cell derived organoids resembling the parts of human cortico-striatal-thalamo-cortical circuit

2024-10-16 | GSE279264 | GEO

In vivo cell-autonomous transcriptional abnormalities revealed in mice expressing mutant huntingtin in striatal but not cortical neurons

Project description:Huntington’s disease (HD), caused by a CAG repeat expansion in the huntingtin (HTT) gene, is characterized by abnormal protein aggregates and motor and cognitive dysfunction. Htt protein is ubiquitously expressed, but the striatal medium spiny neuron (MSN) is most susceptible to neuronal dysfunction and death. Abnormal gene expression represents a core pathogenic feature of HD, but the relative roles of cell-autonomous and non-cell-autonomous effects on transcription remain unclear. To determine the extent of cell-autonomous dysregulation in the striatum in vivo, we examined genome-wide RNA expression in symptomatic D9-N171-98Q (a.k.a. DE5) transgenic mice in which the forebrain expression of the first 171 amino acids of human Htt with a 98Q repeat expansion is limited to MSNs. Microarray data generated from these mice were compared to those generated on the identical array platform from a pan-neuronal HD mouse model, R6/2, carrying two different CAG repeat lengths, and a relatively high degree of overlap of changes in gene expression was revealed. We further focused on known canonical pathways associated with excitotoxicity, oxidative stress, mitochondrial dysfunction, dopamine signaling and trophic support, among others. While genes related to excitotoxicity, dopamine signaling and trophic support, were altered in both DE5 and R6/2 transgenic mice, which may be either cell-autonomous or non-cell-autonomous,, genes related to mitochondrial dysfunction, oxidative stress and the peroxisome proliferator-activated receptor are primarily affected in DE5 transgenic mice, indicating cell autonomous mechanisms Overall, our results demonstrate that HD-induced dysregulation of the striatal transcriptome can be largely attributed to intrinsic effects of mutant Htt,,such that mutant Htt-induced effects in cortical neurons is not necessary for striatal dysfunction/degeneration. Striatum from DE5 transgenic mice vs. wt littermate controls

2011-01-01 | E-GEOD-25232 | biostudies-arrayexpress

Human cortical nuclei RNAseq

Project description:Human cortical nuclei RNAseq

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Human Cortical Kidney Sections

Project description:Human Cortical Kidney Sections

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Human striatal organoids derived from pluripotent stem cells recapitulate striatal development and compartments

Project description:Human striatal organoids derived from pluripotent stem cells recapitulate striatal development and compartments

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FANSseq profiling of human striatal cell types

Project description:FANSseq profiling of human striatal cell types

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Loss of Protocadherin-12 Leads to Diencephalic-Mesencephalic Junction Dysplasia Syndrome.

Project description:ObjectiveTo identify causes of the autosomal-recessive malformation, diencephalic-mesencephalic junction dysplasia (DMJD) syndrome.MethodsEight families with DMJD were studied by whole-exome or targeted sequencing, with detailed clinical and radiological characterization. Patient-derived induced pluripotent stem cells were derived into neural precursor and endothelial cells to study gene expression.ResultsAll patients showed biallelic mutations in the nonclustered protocadherin-12 (PCDH12) gene. The characteristic clinical presentation included progressive microcephaly, craniofacial dysmorphism, psychomotor disability, epilepsy, and axial hypotonia with variable appendicular spasticity. Brain imaging showed brainstem malformations and with frequent thinned corpus callosum with punctate brain calcifications, reflecting expression of PCDH12 in neural and endothelial cells. These cells showed lack of PCDH12 expression and impaired neurite outgrowth.InterpretationDMJD patients have biallelic mutations in PCDH12 and lack of protein expression. These patients present with characteristic microcephaly and abnormalities of white matter tracts. Such pathogenic variants predict a poor outcome as a result of brainstem malformation and evidence of white matter tract defects, and should be added to the phenotypic spectrum associated with PCDH12-related conditions. Ann Neurol 2018;84:646-655.

| S-EPMC6510237 | biostudies-literature

FANSseq profiling of human striatal cell types

Project description:Selective degeneration of striatal projection neurons is one of the main features of Huntington’s disease (HD). The differences between vulnerable and resilient striatal cell types and cell type-specific molecular events associated with HD progression have not been established. Here we employed fluorescence-activated nuclear sorting (FANS) and deep molecular profiling to gain insight into the properties of different cell types of the human striatum in HD and control donors.

2023-12-04 | GSE227729 | GEO

Ventral mesencephalic dopaminergic (vmDA) precursors derived from human embryonic stem cells

Project description:Ventral mesencephalic dopaminergic (vmDA) precursors derived from human embryonic stem cells

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GNPS - Human NVU - Cortical Astrocytes

Project description:Cortical human fetal astrocytes, density 8-15000 cells/cm2 ( ScienCell, San Diego, CA, USA) seeded in Astrocyte medium (ScienCell, San Diego, CA, USA). Further info in "Proteomic and metabolomic characterization of human neurovascular unit cells in response to methamphetamine" Herland et al Adv Biosystems 2020

2020-07-23 | MSV000085817 | GNPS

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