Most recent studies have found that the occurrence and progression of SFTs result from mutual fusion of the NAB2
and STAT6 genes due to internal rearrangement of the 12q13 chromosome, and the most common fusion variants are NAB2ex4 and STAT6ex2/3.
Milbrandt, "NAB2, a corepressor of NGFI-A (Egr-1) and Krox20, is induced by proliferative and differentiative stimuli," Molecular and Cellular Biology, vol.
Svaren, "NAB2 represses transcription by interacting with the CHD4 subunit of the nucleosome remodeling and deacetylase (NuRD) complex," Journal of Biological Chemistry, vol.
Notable upregulated genes were involved in neuron differentiation ( BTG2, NTNG2, DLL1, HES1, NR4A2, STAT3, and VEGF-A ), myeloid cell and osteoclast differentiation ( KLF10, TOB2, and ZFP36 ), cellular and macromolecular biosynthetic process ( KLF13, MKL1, HES1, MYOG, NR4A1, NR4A2, STAT3, and MAF ), and skeletal system development (JUND, KLF10, NAB2
, MYOG, and TIPARP ).
Bellone et al., "Search for mutations in the EGR2 corepressor proteins, NAB1 and NAB2
, in human peripheral neuropathies," Neurogenetics, vol.
Johnson, "EGR1, EGR2, and EGR3 activate the expression of their coregulator NAB2
establishing a negative feedback loop in cells of neuroectodermal and epithelial origin," Journal of Cellular Biochemistry, vol.
(37,38) In solitary fibrous tumors, signal transducer and activator of transcription 6 (STAT6) IHC is, again, highly predictive of the pathognomonic NGFI-A binding protein 2 (NAB2
is a transcriptional corepressor, a regulator of the early growth response 1 (EGR1) transcription factor.