Telomerase regulation in hematological cancers: a matter of stemness?
Human telomerase is a nuclear ribonucleoprotein enzyme complex that catalyzes the synthesis and extension of telomeric DNA. This enzyme is highly expressed and active in most malignant tumors while it is usually not or transiently detectable in normal somatic cells, suggesting that it plays an important role in cellular immortalization and tumorigenesis. As most leukemic cells are generally telomerase-positive and have often shortened telomeres, our understanding of how telomerase is deregulated in these diseases could help to define novel therapies targeting the telomere/telomerase complex. Nonetheless, considering that normal hematopoietic stem cells and some of their progeny do express a functional telomerase, it is tempting to consider such an activity in leukemias as a sustained stemness feature and important to understand how telomere length and telomerase activity are regulated in the various forms of leukemias.
Deville
Laure
L
INSERM UMR-S 685, Institut d'Hématologie, Hôpital Saint-Louis, 75475 Paris cedex 10, France.
Hillion
Josette
J
Ségal-Bendirdjian
Evelyne
E
D000818:Animals; D002471:Cell Transformation, Neoplastic / Q000378:metabolism*; D004273:DNA, Neoplasm / Q000378:metabolism; D015973:Gene Expression Regulation, Leukemic; D015972:Gene Expression Regulation, Neoplastic; D019337:Hematologic Neoplasms / Q000188:drug therapy / Q000201:enzymology*; D006801:Humans; D007938:Leukemia / Q000201:enzymology*; D009363:Neoplasm Proteins / Q000037:antagonists & inhibitors / Q000096:biosynthesis*; D019098:Telomerase / Q000037:antagonists & inhibitors / Q000096:biosynthesis*; D016615:Telomere / Q000378:metabolism
D004273:DNA, Neoplasm; D009363:Neoplasm Proteins; D019098:Telomerase