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Omics · study · 2026

Hypomorphic Lig4 gene mutation in mice predisposes to Th1-skewing intestinal inflammation

Listed in NCBI GEO

Dysregulation of DNA double-strand break (DSB) repair leads to adaptive immunodeficiency, while the remaining lymphocytes are aberrantly activated and provoke inflammation.

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To clarify the biological impact of the LIG4 W447C variant identified in our patient, we generated the mutant mice of DNA ligase IV (LIG4), which is critical for DSB repair. Lig4_W447C/W447C mice showed severe intestinal inflammation under adaptive immunodeficiency.

The inflammation was featured by a marked infiltration of T helper type 1 (Th1) cells and macrophages. We used scRNA-seq to analyse the detailed splenocyte population and T cell receptor repertoire in Lig4_W447C/W447C mice. Evaluation of the T cell receptor repertoire in Lig4_W447C/W447C mice revealed activated oligoclonal Th1 cells with restricted Vα diversity.

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Thus, our present results with our novel hypomorphic Lig4 mutant mice show that defective DSB repair leads to Th1-dependent intestinal inflammation under severe adaptive immunodeficiency.

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Life Sciences
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Single-cell RNA sequencing 65%
Provenance · 1 source records, 8 field assertions
SourceKeyLast seenRaw
NCBI GEOGSE29416915 d agoJSON v1
FieldAssertionExtractorEvidence
access_levelsource · NCBI GEOconnector:ncbi_geo@1.0.0
concepts[field].local:field:life-sciencesmapping · NCBI GEOconnector:ncbi_geo@1.0.0
concepts[method].geo_series_type:expression-profiling-by-high-throughput-sequencingsource · NCBI GEOconnector:ncbi_geo@1.0.0/gdstype
concepts[modality].local:modality:single-cell-rna-seqenrichment · NCBI GEOkeyword-concept-rules@1.0.0title+description (65%)
concepts[organism].NCBITaxon:10090source · NCBI GEOconnector:ncbi_geo@1.0.0/taxon
descriptionsource · NCBI GEOconnector:ncbi_geo@1.0.0/summary
publication_datesource · NCBI GEOconnector:ncbi_geo@1.0.0
titlesource · NCBI GEOconnector:ncbi_geo@1.0.0/title