Editorial Acute promyelocytic leukemia (APL), once a rapidly fatal disease, has become most curable of all leukemias, thanks to the two Chinese wonder drugs, all-trans retinoid acid and arsenic trioxide (AT). PML-RXARa fusion protein is the hallmark and driving force of APL. In the April 9 issue of the journal, Science, Zhang et al. provided new and convincing evidence on how AT, an ancient poi...

The ubiquitin-like SUMO proteins covalently modify protein substrates and regulate their functional properties. In a broad spectrum of cancers, the tumor suppressor PML undergoes ubiquitin-mediated degradation primed by CK2 phosphorylation. Here, we report that the SUMO E3-ligase inhibitor PIAS1 regulates oncogenic signaling through its ability to sumoylate PML and the PML-RARA oncoprotein of a...

The ubiquitin-like SUMO proteins covalently modify protein substrates and regulate their functional properties. In a broad spectrum of cancers, the tumor suppressor PML undergoes ubiquitin-mediated degradation primed by CK2 phosphorylation. Here, we report that the SUMO E3-ligase inhibitor PIAS1 regulates oncogenic signaling through its ability to sumoylate PML and the PML-RARA oncoprotein of a...

BACKGROUND Acute promyelocytic leukemia (APL) can be life threatening, necessitating emergency therapy with prompt diagnosis by morphologic findings, immunophenotyping, cytogenetic analysis, or molecular studies. This study aimed to assess the current routine practices in APL and the clinico-pathologic features of APL. METHODS We reviewed the medical records of 48 Korean patients (25 men, 23 ...

Acute promyelocytic leukemia (APL) is an interesting model for cancer research because of the presence of the specific PML-RARa fusion gene associated with the clinical response t o retinoic acid differentiation therapy. To better understand and improve differentiation induction with retinoic acid, we have established a human APL-ascites model in SCID mice using the NB4 human APL cell line. NB4...

Acute promyelocytic leukemia (APL) occurs in about 530% of adult de novo AML and is characterized by the presence of the chromosomal translocation t(15;17)(q24;q21). The resulting PML-RARA chimeric gene involves the retinoic acid receptor alpha-(RARA) gene on chromosome 17 and the PML gene, a putative transcription factor, on chromosome 15. The resulting PMLRARA chimeric protein is crucial to t...

Balanced chromosomal translocations that generate chimeric oncoproteins are considered to be initiating lesions in the pathogenesis of acute myeloid leukemia. The most frequent is the t(15;17)(q22;q21), which fuses th leukemia (APL). An increasing proportion of APL cases are therapy develop following exposure to radiotherapy and/or chemotherapeutic agents that target DNA topoisomerase II (topoI...

1. Abstract 2. Introduction 2.1. The PML gene 2.2. PML nuclear bodies (PML-NBs) 3. PML-NB disruption and acute promyelocytic leukemia (APL) 3.1. t (15;17) associated APL and PML-NB disruption 3.2. Alternative APL Fusions 3.3. Homodimerization of the APL fusion proteins 3.4. Is forced dimerization of RARA and transcriptional repression sufficient for leukemogenesis? 4. Evidence supporting PML-NB...

The treatment of acute promyelocytic leukemia (APL) patients who present or develop resistance to ATRA is still a problem. Recently, we proposed a pulsed-ATRA regimen, able to reduce the incidence of resistance if compared to continuous administration, and to reinduce complete molecular remission in relapsed patients. Arsenic trioxide (As2O3) is effective in APL refractory or relapsed after ATR...

introduction: arsenic trioxide is effective and approved for treatment of relapsed or refractory apl cases to atra but its effects in new cases of apl is not clear and needs long term follow up to dis close the role of this drug in treatment of apl in combination with chemotherapy/atra or alone. material and methods: we studied 111cases of apl (94 new case and 17 relapsed) diagnosed by mor phol...