| General information | Literature | Expression | Regulation | Mutation | Interaction |
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Basic Information |
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|---|---|
Gene ID | 678 |
Name | ZFP36L2 |
Synonymous | BRF2|ERF-2|ERF2|RNF162C|TIS11D;ZFP36 ring finger protein-like 2;ZFP36L2;ZFP36 ring finger protein-like 2 |
Definition | EGF-response factor 2|ZFP36-like 2|butyrate response factor 2|zinc finger protein 36, C3H type-like 1|zinc finger protein 36, C3H type-like 2|zinc finger protein 36, C3H1 type-like 2|zinc finger protein, C3H type, 36-like 2 |
Position | 2p22.3-p21 |
Gene type | protein-coding |
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Title |
Abstract |
| Deletion of the RNA-binding proteins ZFP36L1 and ZFP36L2 leads to perturbed thymic development and T lymphoblastic leukemia. | ZFP36L1 and ZFP36L2 are RNA-binding proteins (RBPs) that interact with AU-rich elements in the 3 untranslated region of mRNA, which leads to mRNA degradation and translational repression. Here we show that mice that lacked ZFP36L1 and ZFP36L2 during thymopoiesis developed a T cell acute lymphoblastic leukemia (T-ALL) dependent on the oncogenic transcription factor Notch1. Before the onset of T-ALL, thymic development was perturbed, with accumulation of cells that had passed through the beta-selection checkpoint without first expressing the T cell antigen receptor beta-chain (TCRbeta). Notch1 expression was higher in untransformed thymocytes in the absence of ZFP36L1 and ZFP36L2. Both RBPs interacted with evolutionarily conserved AU-rich elements in the 3 untranslated region of Notch1 and suppressed its expression. Our data establish a role for ZFP36L1 and ZFP36L2 during thymocyte development and in the prevention of malignant transformation. |