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Open AccessJournal ArticleDOI

Inherited GATA3 variants are associated with Ph-like childhood acute lymphoblastic leukemia and risk of relapse

TLDR
In a genome-wide association study of 511 ALL cases and 6,661 non-ALL controls, a susceptibility locus for Ph-like ALL is identified and genotype at the GATA3 SNP was associated with early treatment response and risk of ALL relapse, providing insights into interactions between inherited and somatic variants and their role in ALL pathogenesis and prognosis.
Abstract
Recent genomic profiling of childhood acute lymphoblastic leukemia (ALL) identified a high-risk subtype with an expression signature resembling that of Philadelphia chromosome-positive ALL and poor prognosis (Ph-like ALL). However, the role of inherited genetic variation in Ph-like ALL pathogenesis remains unknown. In a genome-wide association study (GWAS) of 511 ALL cases and 6,661 non-ALL controls, we identified a susceptibility locus for Ph-like ALL (GATA3, rs3824662; P = 2.17 × 10(-14), odds ratio (OR) = 3.85 for Ph-like ALL versus non-ALL; P = 1.05 × 10(-8), OR = 3.25 for Ph-like ALL versus non-Ph-like ALL), with independent validation. The rs3824662 risk allele was associated with somatic lesions underlying Ph-like ALL (CRLF2 rearrangement, JAK gene mutation and IKZF1 deletion) and with variation in GATA3 expression. Finally, genotype at the GATA3 SNP was also associated with early treatment response and risk of ALL relapse. Our results provide insights into interactions between inherited and somatic variants and their role in ALL pathogenesis and prognosis.

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A causal mechanism for childhood acute lymphoblastic leukaemia.

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Genome-wide association studies of cancer: current insights and future perspectives

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References
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Journal ArticleDOI

A common JAK2 haplotype confers susceptibility to myeloproliferative neoplasms

TL;DR: A haplotype is identified that preferentially acquires JAK2V617F and confers susceptibility to myeloproliferative neoplasms, suggesting that a certain combination of SNPs may render haplotypes differentially susceptible to somatic mutagenesis.
Journal ArticleDOI

Discovering hematopoietic mechanisms through genome-wide analysis of GATA factor chromatin occupancy.

TL;DR: Fundamental principles underlying GATA factor mechanisms in chromatin are established and a complex network of considerable importance for the control of hematopoiesis is illustrated.
Journal ArticleDOI

Genome-wide Analyses of Transcription Factor GATA3-Mediated Gene Regulation in Distinct T Cell Types

TL;DR: Overall, although GATA3 binding exhibited both shared and cell-specific patterns among various T cell lineages, many genes were either positively or negatively regulated by GATA2 in a cell type-specific manner, suggesting that Gata3-mediated gene regulation depends strongly on cofactors existing in different T cells.
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