GEO series
Muscle Exosome-Derived miR-126 Regulates Local Synthesis of TDP-43 and NMJ Integrity in ALS
GSE286913
Mus musculus (mouse)
Non-coding RNA profiling
18 samples
Submitted 2025/07/15
Platform GPL24247
At a glance
Non-coding RNA profiling · mouse · 18 samples
- What it is
- Non-coding RNA profilingLevels of RNAs that do not code for proteins, such as miRNA or lncRNA.
- Organism
- Mus musculus (mouse)
- Samples
- 18 samples: 6 conditions, mostly in triplicateGroups are read from the sample names, not from the paper. Check the list below.
- 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.
- Paper
- Muscle-derived miR-126 regulates TDP-43 axonal local synthesis and NMJ integrity in ALS modelsNature neuroscience · 2025
Summary (written by the submitters)
Amyotrophic lateral sclerosis (ALS) is characterized by neuromuscular junction (NMJ) disruption and neurodegeneration. Recent findings highlight a pivotal role for TDP-43 in forming axonal pathological condensates and facilitating NMJ disruption through inhibition of local protein synthesis. However, the mechanisms which drive local TDP-43 accumulation remain unknown.
Read the full summary ↓
Here we identify TDP-43 axonal accumulation in peripheral nerves of SOD1 patients and mice stemming from its aberrant local synthesis. This is a non-cell-autonomous process driven by muscle-derived miR-126a-5p exosomes. Inhibiting muscle secretion of miR-126a-5p prompts pre-synaptic TDP-43 synthesis and accumulation that disrupts axonal translation and causes NMJ degeneration. Introducing miR-126 to SOD1G93A mice, primary co-cultures, and human iPSC-derived co-cultures with ALS mutations exhibits neuroprotective effects and delays motor decline. These findings identify a transcellular communication axis between muscles and motor neurons that regulates axonal local synthesis and NMJ maintenance offering insights into ALS onset and progression.
Published in
Ionescu A, Ankol L, Ganapathy Subramaniam A et al.
· Nature neuroscience 2025
· PMID 41044342
· doi:10.1038/s41593-025-02062-6
This dataset
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.
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 GSE286913_RAW.tar (it can run to tens of gigabytes).
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.
Also filed as BioProject PRJNA1210537 and SRA study SRP557302. Searching any of these in the dataset finder brings you back here.
Study design
6 conditions, mostly in triplicate
miRNA-seq of whole cell lysate from primary m… ×3
miRNA-seq of EVs isolated from primary muscle… ×3
miRNA-seq of motor neuron axons treated with … ×3
miRNA-seq of motor neuron axons treated with … ×3
mRNA-seq of motor neuron axons treated with v… ×3
mRNA-seq of motor neuron axons treated with p… ×3
Supports a between-group comparison across 18 samples.
How we read this: 6 replicated groups read from 18 sample titles; they account for 18 of them. These groups are guessed from the sample names, not taken from the paper, so check them against the sample list below before relying on them.
Samples in this study
- GSM8733698 miRNA-seq of whole cell lysate from primary muscle cultures, rep 1 miRNA-seq of whole cell…
- GSM8733699 miRNA-seq of whole cell lysate from primary muscle cultures, rep 2 miRNA-seq of whole cell…
- GSM8733700 miRNA-seq of whole cell lysate from primary muscle cultures, rep 3 miRNA-seq of whole cell…
- GSM8733701 miRNA-seq of EVs isolated from primary muscle cultures conditioned medium, rep 1 miRNA-seq of EVs isolat…
- GSM8733702 miRNA-seq of EVs isolated from primary muscle cultures conditioned medium, rep 2 miRNA-seq of EVs isolat…
- GSM8733703 miRNA-seq of EVs isolated from primary muscle cultures conditioned medium, rep 3 miRNA-seq of EVs isolat…
- GSM8733704 miRNA-seq of motor neuron axons treated with vehicle, rep 1 miRNA-seq of motor neur…
- GSM8733705 miRNA-seq of motor neuron axons treated with vehicle, rep 2 miRNA-seq of motor neur…
- GSM8733706 miRNA-seq of motor neuron axons treated with vehicle, rep 3 miRNA-seq of motor neur…
- GSM8733707 miRNA-seq of motor neuron axons treated with primary muscle EVs, rep 1 miRNA-seq of motor neur…
- GSM8733708 miRNA-seq of motor neuron axons treated with primary muscle EVs, rep 2 miRNA-seq of motor neur…
- GSM8733709 miRNA-seq of motor neuron axons treated with primary muscle EVs, rep 3 miRNA-seq of motor neur…
- GSM8733710 mRNA-seq of motor neuron axons treated with vehicle, rep 1 mRNA-seq of motor neuro…
- GSM8733711 mRNA-seq of motor neuron axons treated with vehicle, rep 2 mRNA-seq of motor neuro…
- GSM8733712 mRNA-seq of motor neuron axons treated with vehicle, rep 3 mRNA-seq of motor neuro…
- GSM8733713 mRNA-seq of motor neuron axons treated with primary muscle EVs, rep 1 mRNA-seq of motor neuro…
- GSM8733714 mRNA-seq of motor neuron axons treated with primary muscle EVs, rep 2 mRNA-seq of motor neuro…
- GSM8733715 mRNA-seq of motor neuron axons treated with primary muscle EVs, rep 3 mRNA-seq of motor neuro…
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Share this dataset
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.