A previously uncharacterized gene, PML, is fused to the retinoic acid receptor alpha gene in acute promyelocytic leukaemia.
It has become apparent that a number of genes disrupted by chromosomal rearrangements during leukaemogenesis encode protein factors involved in transcriptional regulation (20-25) and PML may be another such gene. The replacement of the NH2-terminal transactivation domain in RAR alpha by the PML putative DNA binding and transactivation domains in the PML/RARA fusion produces a novel chimeric protein which may act to block normal myeloid differentiation through disregulation of the genes normally regulated by either or both of the normal proteins.
Goddard
A D
AD
Somatic Cell Genetics Laboratory, Imperial Cancer Center Research Fund, London, United Kingdom.
Borrow
J
J
Solomon
E
E
D002352:Carrier Proteins / Q000235:genetics*; D002884:Chromosomes, Human, Pair 15; D002886:Chromosomes, Human, Pair 17; D015321:Gene Rearrangement; D006801:Humans; D015473:Leukemia, Promyelocytic, Acute / Q000235:genetics*; D009363:Neoplasm Proteins / Q000235:genetics*; D018168:Receptors, Retinoic Acid; D014157:Transcription Factors / Q000235:genetics; D014178:Translocation, Genetic / Q000235:genetics*; D016335:Zinc Fingers / Q000235:genetics
D002352:Carrier Proteins; D009363:Neoplasm Proteins; D018168:Receptors, Retinoic Acid; D014157:Transcription Factors