How Genomic and Structural Context Could Shape JAK-STAT Variant Pathogenicity

The Janus kinase (JAK)-Signal Transducer and Activator of Transcription (STAT) pathway is essential for cellular signal transduction, regulating immune responses, hematopoiesis, and cell proliferation. Dysregulation of JAK-STAT signaling due to genetic variations, particularly missense mutations, has been implicated in autoimmune disorders, cancers, and hematological malignancies. This study investigates missense mutations in JAK and STAT genes, focusing on disease-associated single nucleotide polymorphisms (SNPs) and ClinVar benign variants identified in the All of Us and COSMIC databases. We analyzed the distribution of these mutations across functional domains, their structural localization, and biochemical properties. We identified mutation hotspots within specific domains, highlighting their correlation with disease phenotypes. Structural mapping revealed that disease-associated SNPs predominantly localize in linker regions and at the boundaries of secondary structures, suggesting a significant impact on folding, stability, and function of JAK and STAT proteins. Additionally, we examined the genomic context of mutations and identified vulnerable sequences; for example, 'GATC'. Furthermore, our analysis found no predominant association between potential CRISPR-Cas9 target sites and ClinVar benign/disease-associated SNPs. The analysis of amino acid sequence patterns surrounding mutations uncovered an enrichment of hydrophobic residues leucine (Leu), isoleucine (Ile), methionine (Met), and phenylalanine (Phe) in close proximity to disease-associated mutations. Our findings emphasize the importance of structural and biochemical context in determining pathogenicity. In this study, we provide a bioinformatic strategy for refining variant classification and understanding the roles of JAK-STAT pathway mutations in disease.

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Publication Details

Journal
Twin Research and Human Genetics
Published
2026-03-31
DOI
https://doi.org/10.1017/thg.2026.10054
Primary Topic
Cytokine Signaling Pathways and Interactions
Type
article
Field-Weighted Citation Impact
0.00

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article

How Genomic and Structural Context Could Shape JAK-STAT Variant Pathogenicity

Hye Kyung Lee, Markus Hoffmann
Twin Research and Human Genetics
Cytokine Signaling Pathways and Interactions
article

How Genomic and Structural Context Could Shape JAK-STAT Variant Pathogenicity

Hye Kyung Lee, Markus Hoffmann
article en

Abstract

The Janus kinase (JAK)-Signal Transducer and Activator of Transcription (STAT) pathway is essential for cellular signal transduction, regulating immune responses, hematopoiesis, and cell proliferation. Dysregulation of JAK-STAT signaling due to genetic variations, particularly missense mutations, has been implicated in autoimmune disorders, cancers, and hematological malignancies. This study investigates missense mutations in JAK and STAT genes, focusing on disease-associated single nucleotide polymorphisms (SNPs) and ClinVar benign variants identified in the All of Us and COSMIC databases. We analyzed the distribution of these mutations across functional domains, their structural localization, and biochemical properties. We identified mutation hotspots within specific domains, highlighting their correlation with disease phenotypes. Structural mapping revealed that disease-associated SNPs predominantly localize in linker regions and at the boundaries of secondary structures, suggesting a significant impact on folding, stability, and function of JAK and STAT proteins. Additionally, we examined the genomic context of mutations and identified vulnerable sequences; for example, 'GATC'. Furthermore, our analysis found no predominant association between potential CRISPR-Cas9 target sites and ClinVar benign/disease-associated SNPs. The analysis of amino acid sequence patterns surrounding mutations uncovered an enrichment of hydrophobic residues leucine (Leu), isoleucine (Ile), methionine (Met), and phenylalanine (Phe) in close proximity to disease-associated mutations. Our findings emphasize the importance of structural and biochemical context in determining pathogenicity. In this study, we provide a bioinformatic strategy for refining variant classification and understanding the roles of JAK-STAT pathway mutations in disease.

Twin Research and Human Genetics
National Institutes of Health (US), Georgetown University (US), Georgetown University Medical Center (US)
National Institutes of Health, National Institute of Diabetes and Digestive and Kidney Diseases
Openalex Percentile: Top 31%
Cytokine Signaling Pathways and Interactions
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