TEL-JAK2

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TEL-JAK2 is a gene fusion resulting from a t(9;12) chromosomal translocation detected in cases of human leukemia (Lacronique et al, 1997; Peeters et al, 1997). The TEL gene (also called ETV6) encodes a transcription factor of the ETS family. The TEL protein contains an oligomerisation domain, and its presence in the TEL-JAK2 protein leads to JAK2 juxtaposition and constitutive activation.

The strong transforming potential of constitutively active TEL-JAK2 has been demonstrated by in vitro and in vivo approaches:

- TEL-JAK2 expression, or any other TEL-JAK fusion, renders the Ba/F3 pro-B cell line interleukin 3-independent (Lacronique et al, 2000; Schwaller et al, 1998);

- bone marrow cells retrovirally transduced with TEL-JAK2 originate a lympho/myeloproliferative disorder after transplantation into irradiated mice (Schwaller et al, 1998);

- and TEL-JAK2 transgenic expression in lymphoid cells results in the development of highly penetrant clonal or oligoclonal T-cell leukemia/lymphoma in mice.

References

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