GEO series

Innervation and cargo-specific axonal transport impairments in FUS-ALS mice with gain and loss of function [RNA-seq]

GSE298456 Mus musculus (mouse) RNA-seq (gene expression) 27 samples Submitted 2025/05/29 Platform GPL24247

At a glance

RNA-seq (gene expression) · mouse · 27 samples

What it is: RNA-seq (gene expression)Gene activity measured by sequencing. The summary does not mention single-cell work, so this is most likely bulk: one average per sample, the usual starting point for comparing groups.
Organism: Mus musculus (mouse)
Samples: 27 samples
Data you can get: A processed table (series matrix) and supplementary files from NCBI; the raw sequencing reads from ENA. Which of these hold counts depends on the study, so check the file list.

Summary (written by the submitters)

Mutations in the RNA-binding protein FUS cause aggressive, early-onset forms of amyotrophic lateral sclerosis (ALS), leading to the progressive degeneration of the neuromuscular system. These mutations drive nuclear depletion and cytoplasmic mislocalisation of FUS, resulting in both loss and gain of function. We investigated these mechanisms and their impact on key pathophysiological processes in vivo, using a novel FUS-ALS mouse model.

Read the full summary ↓

Adult mice presented widespread transcriptomic alterations consistent with nuclear loss of function, while translatome analysis revealed specific deficits associated with aberrant FUS-dependent cytoplasmic partitioning and gain of function. Mutant FUS expression selectively impaired neuromuscular junction (NMJ) innervation in FUS-ALS females. However, NMJ reinnervation following sciatic nerve crush was equally perturbed in homozygous mutants of both sexes, indicating impaired regeneration and damage responses. Additionally, we observed cargo-specific alterations in axonal transport, a process critical for neuronal function and maintenance; in vivo mitochondrial transport was impaired in homozygous FUS-ALS mice, whereas the retrograde transport of signalling endosomes was again only affected in mutant females, strengthening the link between endosomal transport impairment and NMJ denervation in ALS. Altogether, our findings establish FUS-ALS homozygous mice as a valuable model for investigating early cellular impairments driving ALS pathology, and identify broader and sex-dependent motor neuron dysfunction in FUS-ALS pathogenesis.

This dataset

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Download · which file do I need?

Direct links to NCBI and ENA, no account and no request form. Pick by what you want to do: take the small processed table to look, the supplementary files to re-analyse, or the original raw data to start over.

1 · Just have a look

Processed table

The series matrix: one table of values per sample as the submitters processed it. The smallest download, and it opens in Excel or R. Start here to see what the data look like.

Series matrix ↓

2 · Re-analyse it

Supplementary files

The count tables and other files the authors uploaded. Take only the samples you need, or the whole study as GSE298456_RAW.tar (it can run to tens of gigabytes).

All supplementary files ↗ Whole study · RAW.tar ↓

3 · Start from the raw reads

Raw sequencing reads

The original FASTQ files, straight from the sequencer, held at ENA. Large, and you align them yourself, so only choose this if you need to re-process the study from scratch.

Raw reads on ENA ↗

What the files usually look like: For RNA-seq the counts are usually a table among the supplementary files (look for 'counts', 'expression', 'FPKM' or 'TPM' in the file name). The series matrix of a sequencing study is sometimes nearly empty.

Also filed as BioProject PRJNA1269488 and SRA study SRP588737. Searching any of these in the dataset finder brings you back here.

NCBI GEO page ↗

Samples in this study

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+ 27 more — browse all 27 samples with per-sample file links →

Datasets on a similar topic

GSE298457 Innervation and cargo-specific axonal transport impairments in FUS-ALS mice with gain and loss of function [RiboTag] 20 samples shares fus-als, cargo-specific, innervation, impairments, transport, axonal, gain
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GSE195825 An axonal guidance cue Semaphorin 6D orchestrates systemic metabolism and myelopoiesis through tuning sympathetic innervation 24 samples shares innervation, axonal
GSE336872 Pitx2-associated early-onset glaucoma alters corneal innervation and sensory function in a sex-specific manner [RNA-Seq Trigeminal Ganglion] 20 samples shares innervation, rna-seq
GSE336875 Pitx2-associated early-onset glaucoma alters corneal innervation and sensory function in a sex-specific manner [RNA-Seq cornea] 20 samples shares innervation, rna-seq
GSE201216 An axonal guidance cue Semaphorin 6D orchestrates systemic metabolism and myelopoiesis through tuning sympathetic innervation (epididymal white adipose tissue) 12 samples shares innervation, axonal
GSE286425 A key role of PIEZO2 mechanosensitive ion channel in adipose sensory innervation [scRNA-seq] 3 samples shares innervation, rna-seq
GSE267751 Time-restricted feeding prevents memory impairments induced by obesogenic diet consumption in mice, in part through hippocampal thyroid hormone signaling. 71 samples shares impairments

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Metadata from NCBI GEO, cached and refreshed periodically — the NCBI page above is authoritative. Downloads link straight to NCBI/ENA; nothing is proxied through BioTransfer.