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INTRODUCTION

SUMMARY

Complex feedback pathways regulate the passage of cells through the G1, S, G2, and M phases of the growth cycle. Two key checkpoints control the commitment of cells to replicate DNA synthesis and to mitosis. Many oncogenes and defective tumor-suppressor genes promote malignant change by stimulating cell-cycle entry or disrupting the checkpoint response to DNA damage. Advances in the understanding of genetic and epigenetic mechanisms of gene regulation provide the basis for novel therapeutic approaches. This chapter presents the pathways and the genetic and epigenetic alterations that regulate cell replication and highlights the various oncogenes and tumor-suppressor genes that are involved in hematologic malignancies.

Acronyms and Abbreviations

ALL, acute lymphoid leukemia; AML, acute myelogenous leukemia; APC, anaphase-promoting complex; APL, acute promyelocytic leukemia; ATM, ataxia-telangiectasia mutated; ATR, ATM and Rad3 related; cdc, cell division cycle; cdk, cyclin-dependent kinase; CDKI, cyclin-dependent kinase inhibitor; Chk, checkpoint kinase; CLL, chronic lymphocytic leukemia; CML, chronic myelogenous leukemia; CMML, chronic myelomonocytic leukemia; CTD, carboxy-terminal domain; DDR, DNA damage response; DLBCL, diffuse large B-cell Lymphoma; DSIF, DRB-sensitivity–inducing factor; ER, endoplasmic reticulum; FL, follicular lymphoma; FLAM, flavopiridol, cytarabine, mitoxantrone; GADD, growth arrest and DNA damage; HAT, histone acetyltransferase; HDAC, histone deacetylase; HDACI, histone deacetylase inhibitor; HM, hematologic malignancies; HRa, homologous recombination; HRb, high-risk; Id1, inhibitor of DNA-binding 1; INK4, inhibitor of kinase 4; JAK, Janus-associated kinase; LM, lymphoid malignancies; MAPK, mitogen-activated protein kinase; MCL, mantle cell lymphoma; MDM2, murine double minute protein 2; MDS, myelodysplastic syndromes; ML, malignant lymphoma; MLL, mixed-lineage leukemia; MM, multiple myeloma; MTA, 5¢-deoxy-5¢-(methylthio)adenosine; MTAP, methylthioadenosine phosphorylase; MZL, marginal zone lymphoma; NELF, negative elongation factor; NHEJ, nonhomologous end-joining; NHL, non-Hodgkin lymphoma; N-TEF, negative transcription elongation factor; ODC, ornithine decarboxylase; PCM, plasma cell myeloma; PDGF, platelet derived growth factor; PI3K, phosphatidylinositol 3¢-kinase; PL, prolymphocytic leukemia; PLZF, promyelocytic leukemia Kruppel-like zinc finger; PML, promyelocytic leukemia; P-TEFb, positive transcription elongation factor b; RARα, retinoic acid receptor α; RB, retinoblastoma gene; rPTK, receptor protein-tyrosine kinase; SLL, small lymphocytic lymphoma; R/R, relapsed or refractory; RS, Richter's syndrome; STAT, signal transducer and activator of transcription; TGF-β, transforming growth factor-β; TKI, tyrosine kinase inhibitor; UPR, unfolded protein response.

Mitosis is the final step of a defined program—the cell cycle—that can be separated into four phases: the G1, S, G2, and M phases (Fig. 15–1). A number of surveillance systems (checkpoints) control the cell cycle and interrupt its progression when DNA damage occurs or when cells have failed to complete a necessary event.1 These checkpoints have been given an empirical definition: When the occurrence of event B is dependent on the completion of prior event A, the dependence is a result of a checkpoint if a loss-of-function mutation can be found that relieves the dependence.1 Three major cell-cycle checkpoints have been discovered: the DNA damage checkpoint, the replication checkpoint, and the spindle-pole body duplication checkpoint.2–4 The ...

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