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TIA1-mediated stress granules promote the neurodegeneration of motor neurons through sequestrating mRNA of heat shock protein HSP70 family in C9orf72-poly-GA mice

ABSTRACT: TIA1-mediated stress granules promote the neurodegeneration of motor neurons through sequestrating mRNA of heat shock protein HSP70 family in C9orf72-poly-GA mice

PROVIDER: PRJNA1263502 | ENA |

REPOSITORIES: ENA

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			Action	DRS
	SRR33584519_1.fastq.gz	Fastqsanger.gz
	SRR33584519_2.fastq.gz	Fastqsanger.gz
	SRR33584520_1.fastq.gz	Fastqsanger.gz
	SRR33584520_2.fastq.gz	Fastqsanger.gz
	SRR33584521_1.fastq.gz	Fastqsanger.gz

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TIA1-mediated stress granules promote the neurodegeneration of motor neurons through sequestrating mRNA of heat shock protein HSP70 family in C9orf72-poly-GA mice

Project description:TIA1 knockout in the central nervous system (TIA1Nestin-CKO mice) alleviated motor neuron loss, neuroinflammation and motor dysfunction in C9orf72-poly-GA mice. Mechanistically, we performed RNA-sequencing to find the differentially expressed genes between ALS-WT and ALS-CKO mice.

2025-05-16 | GSE297313 | GEO

Study of TIA1-dependent stress granules in homeostasis (WT) and Welander distal myopathy (WDM)

Project description:Identification of differential protein components between TIA1-dependent stress granules in proteostasis and Welander distal myopathy

2026-01-05 | PXD038028 | Pride

Transcriptome analysis on OVA-GA(10) immunized GA-CFP mice

Project description:The goal of the study is to perform unbiased transcriptome analysis on spinal cord samples of immunized GA-CFP mice to configure the effect of OVA-GA(10) vaccination in a C9orf72 mouse model.

2019-10-04 | GSE138413 | GEO

The expression of Welander distal myopathy-related TIA1 mutation exacerbates the dynamics of P-bodies and stress granules under oxidative stress

Project description:T-cell intracellular antigen 1 (TIA1) is an RNA-binding protein that is primarily involved in the post-transcriptional control of the cellular fate of RNAs. Moreover, it is a key component of stress granules (SGs), RNA and protein aggregates that are formed in response to stressful stimuli to reduce cellular activity as a survival mechanism. TIA1 p.E384K mutation is the genetic cause of Welander distal myopathy (WDM), a late-onset muscular dystrophy whose pathogenesis has been related to modify SGs dynamics. In this study, we have focused on the differential transcriptomic and proteomic characterization of purified wild-type (WT) and WDM TIA1-dependent SGs by affinity immunopurification followed by mass spectrometry and RNA seq analysis under oxidative stress. The results have revealed small differences in specific components of cellular machineries involving in regulating splicing, RNA degradation and/or translation linked to WDM TIA1 expression under oxidative stress. Our findings suggest a potential regulatory linking among WDM TIA1-dependent SGs and the number, size and colocalization/overlapping with P-bodies under oxidative stress. Taken together, these findings allow us to expand our knowledge on the role of Welander’s TIA1 mutation in interfacing SGs and P-bodies dynamics by involving potential regulatory impacts on cell machineries of RNA decay/turnover and/or translation.

2026-01-09 | GSE240735 | GEO

Proximity proteomics of C9orf72 dipeptide repeat proteins

Project description:Profiling of proximity proteomes of C9orf72-derived dipeptide repeat proteins (poly-GA, poly-GR, poly-PR) in HEK293 cells using BioID assay

2021-12-17 | MSV000088581 | MassIVE

A complex of C9ORF72 and p62 uses arginine methylation to eliminate stress granules by autophagy - PART 3

Project description:Mutations in proteins like FUS which cause Amyotrophic Lateral Sclerosis (ALS) result in the aberrant formation of stress granules while ALS-linked mutations in other proteins impede elimination of stress granules. Repeat expansions in C9ORF72, the major cause of ALS, reduce C9ORF72 levels but how this impacts stress granules is uncertain. Here, we demonstrate that C9ORF72 associates with the autophagy receptor p62 and controls elimination of stress granules by autophagy. This requires p62 to associate via the Tudor protein SMN with proteins, including FUS, that are symmetrically arginine-methylated by PRMT5. Mice lacking p62 accumulate arginine-methylated proteins and alterations in FUS-dependent splicing. Finally, patients with C9ORF72 repeat expansions accumulate symmetric arginine dimethylated proteins which co-localize with p62. This suggests that C9ORF72 initiates a cascade of ALS-linked proteins (C9ORF72, p62, SMN, FUS) to recognize stress granules for degradation by autophagy and hallmarks of a defect in this process are observable in ALS patients.

2018-07-25 | PXD009760 | Pride

A complex of C9ORF72 and p62 uses arginine methylation to eliminate stress granules by autophagy - PART 1

2018-07-26 | PXD009759 | Pride

A complex of C9ORF72 and p62 uses arginine methylation to eliminate stress granules by autophagy - PART 2

2018-07-26 | PXD009741 | Pride

GA-CFP data for the manuscript "Correction of dysregulated lipid metabolism normalizes gene expression in oligodendrocytes and prolongs lifespan in female poly-GA C9orf72 mouse model"

Project description:These sequences support the following paper abstract: Clinical and genetic research links altered cholesterol metabolism with ALS development and progression, yet pinpointing specific pathomechanisms remain challenging. We investigated how cholesterol dysmetabolism interacts with protein aggregation, demyelination, and neuronal loss in ALS. Bulk RNAseq transcriptomics showed decreased cholesterol biosynthesis and increased cholesterol export in ALS mouse models (GA-Nes, GA-CFP, rNLS8) and patient samples (spinal cord), suggesting an adaptive response to cholesterol overload. Consequently, we assessed the efficacy of the cholesterol-binding drug 2-hydroxypropyl-β-cyclodextrin (CD) in a fast-progressing C9orf72 ALS mouse model with extensive poly-GA expression and myelination deficits. CD treatment normalized cholesteryl ester levels, lowered neurofilament light chain levels, and prolonged lifespan in female but not male GA-Nes mice, without impacting poly-GA aggregates. Single nucleus transcriptomics indicated that CD primarily affected oligodendrocytes, significantly restored myelin gene expression, increased density of myelinated axons, inhibited the disease-associated oligodendrocyte response, and downregulated the lipid-associated genes Plin4 and ApoD. These results suggest that reducing excess free cholesterol in the CNS could be a viable ALS treatment strategy.

2025-03-08 | GSE289729 | GEO

Correction of dysregulated lipid metabolism normalizes gene expression in oligodendrocytes and prolongs lifespan in female poly-GA C9orf72 mouse model

Project description:These sequences support the following paper abstract: "Clinical and genetic research links altered cholesterol metabolism with ALS development and progression, yet pinpointing specific pathomechanisms remain challenging. We investigated how cholesterol dysmetabolism interacts with protein aggregation, demyelination, and neuronal loss in ALS. Bulk RNAseq transcriptomics showed decreased cholesterol biosynthesis and increased cholesterol export in ALS mouse models (GA-Nes, GA-CFP, rNLS8) and patient samples (spinal cord), suggesting an adaptive response to cholesterol overload. Consequently, we assessed the efficacy of the cholesterol-binding drug 2-hydroxypropyl-β-cyclodextrin (CD) in a fast-progressing C9orf72 ALS mouse model with extensive poly-GA expression and myelination deficits. CD treatment normalized cholesteryl ester levels, lowered neurofilament light chain levels, and prolonged lifespan in female but not male GA-Nes mice, without impacting poly-GA aggregates. Single nucleus transcriptomics indicated that CD primarily affected oligodendrocytes, significantly restored myelin gene expression, increased density of myelinated axons, inhibited the disease-associated oligodendrocyte response, and downregulated the lipid-associated genes Plin4 and ApoD. These results suggest that reducing excess free cholesterol in the CNS could be a viable ALS treatment strategy.”

2025-03-10 | GSE262778 | GEO

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