Cited by 130DNA Repair MechanismsAlzheimer's disease research and treatmentsGenomic variations and chromosomal abnormalities

The DOI matches its Crossref record; 160 of the 162 references it cites were found in a registry or an index; none of the 120 sources its claims cite is checked for support yet.

Neuronal DNA double-strand breaks lead to genome structural variations and 3D genome disruption in neurodegeneration

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Abstract

Persistent DNA double-strand breaks (DSBs) in neurons are an early pathological hallmark of neurodegenerative diseases including Alzheimer's disease (AD), with the potential to disrupt genome integrity. We used single-nucleus RNA-seq in human postmortem prefrontal cortex samples and found that excitatory neurons in AD were enriched for somatic mosaic gene fusions. Gene fusions were particularly enriched in excitatory neurons with DNA damage repair and senescence gene signatures. In addition, somatic genome structural variations and gene fusions were enriched in neurons burdened with DSBs in the CK-p25 mouse model of neurodegeneration. Neurons enriched for DSBs also had elevated levels of cohesin along with progressive multiscale disruption of the 3D genome organization aligned with transcriptional changes in synaptic, neuronal development, and histone genes. Overall, this study demonstrates the disruption of genome stability and the 3D genome organization by DSBs in neurons as pathological steps in the progression of neurodegenerative diseases.

Overview

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Written from the paper’s full text.

TL;DR

Persistent neuronal DNA double-strand breaks are linked to mosaic gene fusions, genome structural variations, and progressive 3D genome disruption in Alzheimer’s disease and mouse neurodegeneration models. These changes are enriched in excitatory neurons with DNA damage/senescence signatures and align with transcriptional changes in synaptic, neuronal development, and histone genes.

Key findings

  • In human postmortem prefrontal cortex snRNA-seq (47 ROSMAP participants; 6,180 cells, 5,421 after QC), gene fusions were enriched in AD pathology (adj. p=0.046) and late AD/Braak 5–6 (adj. p=0.00031), particularly in excitatory neurons; this was validated in 10x snRNA-seq of over 2 million cells from 380 individuals (adj. p<0.001).
  • In CK-p25 mice, Step 2 neurons were enriched for gene fusions (p=5.8e−16); mate-pair sequencing showed structural variation increases of 22% in γH2AX-high neurons, 241% in Step 2 neurons, and 201% after persistent etoposide-induced DSBs, while acute etoposide did not increase structural variations.
  • DSBs were enriched at highly expressed and long genes and at fusion junctions; 25 of 27 recurrent DSB cluster genes appeared in human excitatory neuron fusion calls, and AD GWAS genes were longer and enriched among fusion genes.
  • Hi-C showed reduced long-range interactions, ~8.5% of the genome shifting A/B compartments, decreased TAD inclusion ratios, and reduced loop intensities in Step 1/Step 2 neurons; loop loss correlated with downregulated synaptic and neuronal development genes, while histone gene clusters gained de novo loops alongside histone upregulation.

Methods

  • Human postmortem prefrontal cortex from ROSMAP: Smart-seq2 snRNA-seq in 47 individuals and 10x snRNA-seq in 2,026,710 cells from 380 individuals; gene fusions called with STAR-Fusion and analyzed by negative binomial regression with cell- and individual-level covariates.
  • Mouse and primary neuron models: CK-p25 dox-off induction with FANS gates for NeuN/γH2AX, etoposide-treated primary cortical neurons (acute vs persistent DSBs), and Tau P301S mice; assays included mate-pair sequencing, BLISS, in situ Hi-C, Oxford Nanopore cDNA sequencing, and immunofluorescence/immunohistochemistry.
  • Hi-C analyzed with HOMER/Juicer for compartments, TADs, and loops; gene ontology with GOrilla; human RAD21 staining analyzed with linear mixed-effects models.

Limitations

  • Stated: gene fusions represent only a subset of genome structural variations; scRNA-seq is sparse and likely underestimates fusions per cell; PCR artifacts and random ligations are difficult to distinguish from true mosaic fusions, with the assumption that AD/control classification and expression enrichments do not modify these artifacts; the study cannot resolve whether 3D genome disruption is caused directly by unresolved DSBs or by structural variations.
  • Evident from the text: human findings are cross-sectional associations, and causal inference relies on mouse and etoposide models; the full extent of DNA damage-dependent structural variations would require single-cell long-read genome sequencing.

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Integrity signals

Registry

Crossref · 10.1016/j.cell.2023.08.038

Cites

160 of 160 references verified0 citation errors · 0 not checkable

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Claims the paper makes

63 Own result · 51 Not checked yet · 23 Own interpretation · 12 Own method · 3 No basis given

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  • It may state two separate claims as one. 43
  • Borderline: one of the tests for a substantial claim is unclear. 1
  • The quote only partly states this claim. 3
  • Probably not a substantial claim. 39
73 of 73
  • The authors state this as an assumption.

    Gene fusions detected from snRNA-seq reads are a valid proxy for genome structural variations in neurons.

    “using STAR-fusion to detect chimeric snRNA-seq reads mapping between two genes as a proxy for genome structural variations”
    “increased levels of gene fusions as a proxy for increased genome structural variation”
  • The authors state this as an assumption.

    AD vs. Control classification and various gene expression pattern enrichments explored in this do not modify PCR artifacts and random ligations during library preparation.

    “We make the assumption that AD vs. Control classification and various gene expression pattern enrichments explored in this do not modify PCR artifacts and random ligations during library preparation.”
  • The paper relies on this without support.

    Genome structural variations can bring ectopic regulatory elements next to genes causing pathological up or down-regulation of genes.

    “Genome structural variations can bring ectopic regulatory elements next to genes causing pathological up or down-regulation of genes.”
  • The paper relies on this without support.

    Gene fusions at important neural genes can result in a pathological gain or loss of their function and result in defects in neuronal function.

    “Gene fusions at important neural genes can result in a pathological gain or loss of their function and result in defects in neuronal function.”
  • The paper relies on this without support.

    Gene fusions arise from a subset of genome structural variations.

    “Gene fusions arise from a subset of genome structural variations”
5 of 5

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Metadata

Authors
Dileep, V., Boix, C. A., Mathys, H., Marco, A., Welch, G. M., Meharena, H. S., Loon, A., Jeloka, R., Peng, Z., Bennett, D. A., Kellis, M., & Tsai, L.-H.
Affiliations
  • Massachusetts Institute of Technology (US)
  • MIT Computer Science and Artificial Intelligence Laboratory (US)
  • Rush University Medical Center (US)
  • Broad Institute (US)
Corresponding author
  • Dileep, Vishnu
  • Kellis, Manolis
  • Tsai, Li-Huei
Venue
Cell
Publisher
Elsevier BV
Published
2023-09
Volume
186
Issue
20
Pages
4404-4421.e20
ISSN
0092-8674 (ISSN-L)
OpenAlex
W4387142572
Cited by
130
References
162
Citations by year
2026: 28 · 2025: 53 · 2024: 41 · 2023: 7
FWCI
11.49
Type
journal article
Language
en
Open access
Locations
  • Cell (publishedVersion, cc-by-nc-nd)
  • PubMed (publishedVersion)
  • PubMed Central (acceptedVersion)
Funders
  • NIH (P30AG72975, K99 AG073466, R01AG15819, R01AG17917, P30AG10161, R01 AG058002, U01AG46152, U01 NS110453, U01AG61356, NS051874, AG054012, NS102730)
  • Cure Alzheimer&apos;s Fund
  • Alzheimer&apos;s Association (AARF-19-618751)
  • JPB Foundation
  • Schweizerischer Nationalfonds zur Förderung der Wissenschaftlichen Forschung (R01AG17917, R01AG15819, P30AG72975, P30AG10161, P2BSP3_151885, U01AG61356, U01AG46152)
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › DNA Repair Mechanisms
  • Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Alzheimer's disease research and treatments
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genomic variations and chromosomal abnormalities
Keywords
DNA double-strand breaks, neurodegeneration, Alzheimer's disease, structural variants, 3D genome organization, cohesin, DNA repair, cellular senescence, excitatory neurons, genome stability, histone genes
MeSH terms
Alzheimer Disease, Animals, DNA, DNA Repair, Humans, Neurons, Sequence Analysis, RNA, Neurodegenerative Diseases, Genomic Instability, Mice, DNA Breaks, Double-Stranded, Single-Cell Analysis

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