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      Chromosomal translocation t(15;17) in human acute promyelocytic leukemia fuses RAR alpha with a novel putative transcription factor, PML.

      Cell
      Amino Acid Sequence, Base Sequence, Blotting, Northern, Carrier Proteins, genetics, Chromosomes, Human, Pair 15, Chromosomes, Human, Pair 17, DNA, DNA-Binding Proteins, Gene Expression, Humans, Leukemia, Promyelocytic, Acute, Molecular Sequence Data, Neoplasm Proteins, Nuclear Proteins, Oligonucleotides, chemistry, Oncogenes, Polymerase Chain Reaction, RNA, Messenger, Receptors, Retinoic Acid, Transcription Factors, Transcriptional Activation, Translocation, Genetic, Tretinoin, Tumor Suppressor Proteins

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          Abstract

          A unique mRNA produced in leukemic cells from a t(15;17) acute promyelocytic leukemia (APL) patient encodes a fusion protein between the retinoic acid receptor alpha (RAR alpha) and a myeloid gene product called PML. PML contains a cysteine-rich region present in a new family of apparent DNA-binding proteins that includes a regulator of the interleukin-2 receptor gene (Rpt-1) and the recombination-activating gene product (RAG-1). Accordingly, PML may represent a novel transcription factor or recombinase. The aberrant PML-RAR fusion product, while typically retinoic acid responsive, displays both cell type- and promoter-specific differences from the wild-type RAR alpha. Because patients with APL can be induced into remission with high dose RA therapy, we propose that the nonliganded PML-RAR protein is a new class of dominant negative oncogene product. Treatment with RA would not only relieve this inhibition, but the activated PML-RAR protein may actually promote myelocyte differentiation.

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