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Activation of polo-like kinase 1 expression in spinal motor neurons as vulnerability factor of spinal motor neurons in FUS- and other fALS

ABSTRACT: Activation of polo-like kinase 1 expression in spinal motor neurons as vulnerability factor of spinal motor neurons in FUS- and other fALS

PROVIDER: PRJNA1138888 | ENA |

REPOSITORIES: ENA

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			Action	DRS
	SRR29922779.fastq.gz	Fastqsanger.gz
	SRR29922780.fastq.gz	Fastqsanger.gz
	SRR29922781.fastq.gz	Fastqsanger.gz
	SRR29922782.fastq.gz	Fastqsanger.gz
	SRR29922783.fastq.gz	Fastqsanger.gz

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Activation of polo-like kinase 1 expression in spinal motor neurons as vulnerability factor of spinal motor neurons in FUS- and other fALS

Project description:FUS ALS seems to preferentially affect sMNs, and cognitive dysfunction in FUS ALS is rare. Considering this, we wanted to analyze if cortical neurons behave differently than spinal motor neurons in response to FUS mutations. For this, we used cortical neurons derived from isogenic human induced pluripotent stem cells (hiPSCs) in which either WT or NLS mutant FUS P525L was tagged with eGFP using CRISPR/Cas9 and systematically compared them to sMNs of the identical iPSCs. Phenotypically, mutant FUS cortical neurons showed less impairment of FUS recruitment to DNA damage sites compared to mutant sMNs and less signs of DNA damage, which were similarly found in post mortem tissue. To advance our understanding of finding different molecular mechanisms and pathways related to FUS mutations in ALS disease, we have performed RNA sequencing of FUS ALS cortical and spinal motor neurons and our results revealed basic differences in their transcriptomes. Alternative splicing events in spinal motor neurons were different from cortical neurons also pointing towards DNA damage in FUS ALS.

2024-08-27 | GSE272827 | GEO

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Project description:To clarify the functional properties of FUS, we established the differentially expressed alternative exons in FUS-silenced primary motor neurons by using exon-sensitive microarray technology. To clarify the functional properties of FUS, we established the differentially expressed alternative exons in FUS-silenced primary glial cells by using exon-sensitive microarray technology. To clarify the functional properties of FUS, we established the differentially expressed alternative exons in FUS-silenced primary cerebellar neurons by using exon-sensitive microarray technology.

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2022-05-20 | GSE203168 | GEO

Small RNA-sequencing of FUS H517Q and healthy iPSC-derived motor neurons

Project description:We sought to investigate changes in expression levels of small RNAs including microRNAs in FUS H517Q and healthy motor neurons Motor neurons were differentiated from patient-derived FUS H517Q and healthy iPSCs, and harvested at day 30. We performed gene expression profiling analysis using data obtained from bulk small RNA-seq.

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Changes in FUS interactome in motor neurons upon ALS-related mutation

Project description:FUS Immunoprecipitation in human induced pluripotent stem cells and human motor neurons

2024-03-04 | PXD047271 | Pride

FUS H517Q and healthy iPSC-derived motor neurons

Project description:FUS H517Q and healthy iPSC-derived motor neurons

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Stem cell-derived cranial and spinal motor neurons reveal proteostatic differences between ALS resistant and sensitive motor neurons

Project description:We report the comparative gene expression between embryonic stem cell derived cranial and spinal motor neurons and multiple time points after induction and primary cultured ocular and spinal motor neurons, using single cell RNA sequencing.

2019-07-09 | GSE130938 | GEO

Dynamics of gene expression changes in the spinal cord during FUS-mediated proteinopathy

Project description:Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder leading to the eventual death of motor neurons. Described cases of familial ALS have emerged the significance of protein misfolding and aggregation of two functionally related proteins, FUS and TDP-43, implicated in RNA metabolism. Herein, using the in vivo model of FUS-mediated proteinopathy (FUS1-359 mice) we performed the comprehensive analysis encompassing the onset of the first clinical symptoms, inclusions formations as well as changes in gene expression profile in motor neurons and surrounding microglia. The obtained data show that FUS-mediated proteinopathy is predominantly asymptomatic in terms of both the clinical symptoms and molecular aspects of neurodegeneration until it reaches the terminal stage of disease progression (120 days from birth). From this time point the pathological process develops very rapidly resulting in massive FUS-positive inclusions formation which is accompanying by the transcriptional “burst” in the spinal cord cells. Specifically it manifests in activation of pro-inflammatory phenotype of microglial cells and malfunction of acetylcholine synapse transmission in motor neurons. We conclude that a stable course of the pathological process, as well as described accompanying features, make FUS1-359 mice a convenient model for testing potential therapeutics against proteinopathy-induced decay of motor neurons.

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2023-06-01 | GSE205718 | GEO

Axonal transcriptome reveals upregulation of PLK1 as a protective mechanism in response to increased DNA damage in FUSP525L spinal motor neurons

Project description:Mutations in the gene fused-in-sarcoma (FUS) have been implicated in the motor neuron disease Amyotrophic lateral sclerosis (ALS). However, it is poorly understood how these gene mutations lead to selective motor neuron (MN) degeneration and if there are common pathomechanisms across disease etiology. To address the biological impact of the FUS-P525L mutation on the transcriptome of neurons, we applied RNA-sequencing (RNA-seq) method, using microfluidic chambers, to generate axonal as well as soma compartment specific profiles from isogenic induced pluripotent stem cells (iPSCs)-derived motor neurons. We demonstrate high purity of axonal and soma fractions, and further show that the axonal transcriptome is very specific from somas with fewer number of transcripts. Notably, functional enrichment analysis revealed a unique and distinct transcriptional landscape in both axonal and soma compartments including specific transcript types, most of which were required for RNA metabolism and DNA damage/cell cycle related processes, that were likely altered by FUS mutation. Among altered transcripts, we identified robust upregulation of polo-like kinase 1 (PLK1), a master regulator of cell cycle-related events, in FUS-P525L mutant MNs and confirmed that inhibition of PLK1 increases late apoptotic or necrosis-induced neuronal cell death in mutant neurons compared to healthy controls. Taken together, our findings provide insights into compartment-specific transcriptomics in FUS-ALS neuronal model and we propose that PLK1 activation is a consequence of MN-specific upregulation, possibly contributing to the DNA-damage response and other associated pathways including cell cycle and cytoskeleton machinery.

2024-09-10 | GSE276214 | GEO

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