Abstract
USP6, also known as Tre-2 and TRE17, is an ubiquitase-specific proteinase that was identified more than two decades ago as apotential oncogene when it exhibited transforming properties upon overexpression in NIH 3T3 cells. Until recently, however, little was known about the function and the oncogenetic activation of USP6. The identification of rearrangements of the USP6 gene in aneurysmal bone cyst and in nodular fasciitis has not only led to abetter understanding of the pathogenesis of these entities, but is also auseful tool in their diagnosis and differential diagnostic delineation from morphological mimics. In this review, the clinical, pathomorphological, and molecular genetic aspects of aneurysmal bone cyst and of nodular fasciitis, as well as from related lesions, are presented and discussed.
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