--- bibliography: 'morinlab.bib' csl: 'NLM.csl' link-citations: true nocite: | @viganoSomaticIL4RMutations2018, @dunsCharacterizationDLBCLPMBL2021, --- [[_TOC_]] ## Overview Mutations in IL4R have been identified in various types of B-cell lymphomas, particularly primary mediastinal large B-cell lymphoma (PMBCL) and DLBCL. IL4R is one of [a number of genes](https://github.com/morinlab/LLMPP/wiki/ashm) affected by aberrant somatic hypermutation in B-cell lymphomas, which complicates the interpretation of mutations at this locus. IL4R mutations are found in approximately 24.2% of primary PMBCL cases. These mutations are commonly single nucleotide variants in exon 8, resulting in the I242N amino acid change. This leads to constitutive activation of the JAK-STAT signaling pathway and upregulation of downstream cytokine expression profiles and B cell-specific antigens.[@viganoSomaticIL4RMutations2018; @dunsCharacterizationDLBCLPMBL2021] In DLBCL, IL4R mutations are more rare and tend to occur within the GCB subgroup.[@dunsCharacterizationDLBCLPMBL2021] ## Experimental Evidence Driver mutations affecting this gene in DLBCL have been experimentally demonstrated to cause a gain of function (GOF).[@viganoSomaticIL4RMutations2018] ## Relevance tier by entity [[include:tables/table1_IL4R.md]] ## Mutation incidence in large patient cohorts (GAMBL reanalysis) ### DLBCL [[include:tables/DLBCL_IL4R.md]] ## Mutation pattern and selective pressure estimates [[include:tables/dnds_IL4R.md]] ## aSHM regions |chr_name|hg19_start|hg19_end|region |regulatory_comment| |:--------:|:----------:|:--------:|:------------------------------------------------------------------------------------------:|:------------------:| |chr16 |27322895 |27329423|[TSS](https://genome.ucsc.edu/s/rdmorin/GAMBL%20hg19?position=chr16%3A27322895%2D27329423)|active_promoter | ## IL4R Hotspots | Chromosome |Coordinate (hg19) | ref>alt | HGVSp | | :---:| :---: | :--: | :---: | | chr16 | 27367183 | T>A | I242N | [[include:tables/browser_IL4R.md]] ## Expression  <!-- ORIGIN: viganoSomaticIL4RMutations2018b --> <!-- DLBCL: dunsCharacterizationDLBCLPMBL2021b --> <!-- PMBL: viganoSomaticIL4RMutations2018b --> [[include:tables/mermaid_IL4R.md]] ## References