Читать книгу The Peripheral T-Cell Lymphomas - Группа авторов - Страница 87

EATL is a rare but fatal PTCL arising from the intestinal tract. Genomic alterations in SETD2 gene resulting in SETD2 loss of function, and/or loss of the corresponding 3p.21 locus are found in 31–86% of EATL [46, 47]. SETD2 encodes a non‐redundant H3K36‐specific tri‐methyltransferase that serves as a tumor suppressor. In SETD2‐mutated EATL samples, H3K36me3 expression in tumor specimens was either defective or very weak. To further investigate SETD2 function in T‐cell development, the Setd2 cKO mice were generated with Lck‐Cre transgenic mice [47]. After comparing the proportion of intraepithelial T cells in Setd2 wild‐type versus deficient mice, they observed a significant increase in the population of γδ‐positive T cells in Setd2‐deficient mice. Although these mice do not perfectly recapitulate human EATL, this model may provide a very useful tool for future modeling of this fatal disease and is being evaluated in preclinical studies.