PML
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Definition: PML is a tumour suppressor inactivated in acute promyelocytic leukemia (APL).
Function
PML is the essential component of a subnuclear structure called the PML nuclear body (PML-NB), which is disrupted in APL.
The promyelocytic leukaemia (PML) tumour suppressor protein epitomizes the PML-nuclear body (PML-NB) and is crucially required for the proper assembly of this macromolecular nuclear structure.
Unlike other, more specialized subnuclear structures such as Cajal and Polycomb group bodies, PML-NBs are functionally promiscuous and have been implicated in the regulation of diverse cellular functions.
PML-NBs are dynamic structures that favour the sequestration and release of proteins, mediate their post-translational modifications and promote specific nuclear events in response to various cellular stresses.
PML-NBs may be heterogeneous in composition, mobility and function.
By targeting different cellular proteins to this structure, PML can either hamper or potentiate their functions. The PML transcript undergoes alternative splicing to generate both nuclear and cytoplasmic isoforms.
Pathology
PML gene is fused to the RARalpha gene in the vast majority of acute promyelocytic leukemias (APL) (PML/RARA fusion gene)
PML protein expression is frequently lost in human cancers of various histologic origins, and its loss associates with tumor grade and progression in some tumor histotypes (14970276)
References
Bernardi R, Pandolfi PP. Structure, dynamics and functions of promyelocytic leukaemia nuclear bodies. Nat Rev Mol Cell Biol. 2007 Dec;8(12):1006-16. PMID: 17928811
Salomoni P, Bellodi C. New insights into the cytoplasmic function of PML. Histol Histopathol. 2007 Aug;22(8):937-46. PMID: 17503350