Epigenetic deregulation of Ellis Van Creveld confers robust Hedgehog signaling in adult T-cell leukemia.
Bottom Line: Using microarray, quantitative RT-PCR and immunohistochemistry we have demonstrated that EVC is significantly upregulated in ATL and human T-cell leukemia virus type I (HTLV-1)-infected cells.The HH inhibitors are suggested as drug candidates for ATL therapy.Our findings also suggest chromatin rearrangement associated with active histone markers in ATL.
Affiliation: Graduate School of Frontier Sciences, The University of Tokyo, Tokyo, Japan.Show MeSH
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Mentions: Finally, we evaluated the pharmacological activity of GANT61 on primary ATL samples. Although GANT61 did not show a clear effect on PBMC derived from healthy donors and HTLV-1 carriers, its treatment specifically reduced the viability of ATL samples significantly (Fig.7a). Flow cytometry demonstrated that GANT61 specifically killed CD4+ leukemic cells from ATL patients via apoptosis induction (Fig.7b).
Affiliation: Graduate School of Frontier Sciences, The University of Tokyo, Tokyo, Japan.