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Transcriptomic Characterization Reveals Disrupted Medium Spiny Neuron Trajectories in Huntington's Disease and Possible Therapeutic Avenues

ABSTRACT: Transcriptomic Characterization Reveals Disrupted Medium Spiny Neuron Trajectories in Huntington's Disease and Possible Therapeutic Avenues

PROVIDER: PRJNA973090 | ENA |

REPOSITORIES: ENA

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Transcriptomic Characterization Reveals Disrupted Medium Spiny Neuron Trajectories in Huntington's Disease and Possible Therapeutic Avenues [scRNA-seq]

Project description:Transcriptomic Characterization Reveals Disrupted Medium Spiny Neuron Trajectories in Huntington's Disease and Possible Therapeutic Avenues [scRNA-seq]

| PRJNA973091 | ENA

Transcriptomic Characterization Reveals Disrupted Medium Spiny Neuron Trajectories in Huntington's Disease and Possible Therapeutic Avenues [bulk RNA-seq]

Project description:Transcriptomic Characterization Reveals Disrupted Medium Spiny Neuron Trajectories in Huntington's Disease and Possible Therapeutic Avenues [bulk RNA-seq]

| PRJNA973093 | ENA

Transcriptomic Characterization Reveals Disrupted Medium Spiny Neuron Trajectories in Huntington's Disease and Possible Therapeutic Avenues [scRNA-seq]

Project description:Huntington's Disease (HD) is a neurodegenerative disorder caused by an expansion of CAG repeats in exon 1 of the HTT gene, ultimately resulting in the generation of a mutant HTT (mHTT) protein. Although mHTT is expressed in various tissues, it significantly affects medium spiny neurons (MSNs) in the striatum, resulting in their loss and the subsequent motor function impairment found in HD. While HD symptoms typically emerge in midlife, disrupted MSN neurodevelopment plays an important role. To explore the effects of HD on MSN development, we differentiated induced HD pluripotent stem cells (iPSC) and isogenic control into neuronal stem cells, and then generated a developing MSN population encompassing early, intermediate progenitors, and mature MSNs. Single cell RNA sequencing (scRNAseq) revealed that the developmental trajectory of MSNs in our model closely emulated the trajectory of fetal striatal neurons. However, in the HD MSN cultures, this process was by downregulating several crucial genes for required for proper MSN maturation, including ASCL1 and members of the DLX family of transcription factors. Our analysis also uncovered a progressive dysregulation of multiple HD-related pathways as MSNs matured, including the NRF2-mediated oxidative stress response and ERK/MAPK signaling. Using the transcriptional profile of developing HD MSNs, we searched the L1000 dataset for small molecules that induce an opposite gene expression pattern. Our analysis pinpointed numerous small molecules with known benefits in HD models, as well as previously untested novel molecules. A top novel candidate, Cerulenin, demonstrated a partial restoration of DARPP-32 levels and electrical activity in HD MSNs, while also modulating genes involved in in multiple HD related pathways.

2025-07-22 | GSE232648 | GEO

Transcriptomic Characterization Reveals Disrupted Medium Spiny Neuron Trajectories in Huntington's Disease and Possible Therapeutic Avenues [bulk RNA-seq]

2025-07-22 | GSE232646 | GEO

Transcriptomic Characterization Reveals Disrupted Medium Spiny Neuron Trajectories in Huntington's Disease and Possible Therapeutic Avenues [Bulk RNA-seq treated with Cerulenin]

Project description:Transcriptomic Characterization Reveals Disrupted Medium Spiny Neuron Trajectories in Huntington's Disease and Possible Therapeutic Avenues [Bulk RNA-seq treated with Cerulenin]

| PRJNA973092 | ENA

Transcriptomic Characterization Reveals Disrupted Medium Spiny Neuron Trajectories in Huntington's Disease and Possible Therapeutic Avenues [Bulk RNA-seq treated with Cerulenin]

2025-07-22 | GSE232647 | GEO

Transcriptomic Characterization Reveals Disrupted Medium Spiny Neuron Trajectories in Huntington's Disease and Possible Therapeutic Avenues

Project description:This SuperSeries is composed of the SubSeries listed below.

2025-07-22 | GSE232649 | GEO

RNAseq of FACS Drd1 and Drd2 receptor expressing medium spiny neurons (direct and indirect medium spiny neurons) from 5-6 month BACHD Huntington's disease model mice, compared to WT littermates

Project description:mRNAseq for baseline transcriptional profiling of D1 and D2 receptor-expressing medium spiny neurons from 5-6 month BACHD mice, compared to respective WT littermates. BACHD iSPN and dSPN RNAseq data has been described in "Impaired TrkB receptor signaling underlies corticostriatal dysfunction in Huntington's disease"; Plotkin et al; 2014; PMID accession number 24991961.

2017-08-31 | GSE103341 | GEO

ATAC-Seq for control and huntington's disease iPSC-derived medium spiny neuron-like cells

Project description:ATAC-Seq for control and huntington's disease iPSC-derived medium spiny neuron-like cells

| PRJNA603902 | ENA

Total RNA Sequencing of control and huntington's disease iPSC-derived medium spiny neuron-like cells

Project description:Total RNA Sequencing of control and huntington's disease iPSC-derived medium spiny neuron-like cells

| PRJNA604024 | ENA