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Beschreibung

Transcription factor Spi-B (UniProt: Q01892, also known as SPIB) is encoded by the SPIB gene (Gene ID: 6689) in human. SPIB is a member of the ETS family of transcription factors that is expressed in mature B cells, T-cell progenitors, and in plasmacytoid dendritic cells. It is reported to be a key regulator of human plasmacytoid dendritic cell development. It may also be required for B-cell receptor (BCR) signaling that is essential for normal B-cell development and antigenic stimulation. It serves as a sequence specific transcriptional activator that binds to the PU-box, a purine-rich DNA sequence (5'-GAGGAA-3') that can act as a lymphoid-specific enhancer. SPIB contains a weakly acidic N-terminal transactivation domain (TAD, aa 1-31) that is followed by a second TAD (aa 41-61) that is rich in proline, serine, and threonine. Each of these domains may be required for transcriptional activation of a subset of target genes. Overexpression of SPIB is reported in all cases of blastic plasmacytoid dendritic cell (BPDC) neoplasms, but not in its cutaneous mimics. It can be used as a novel immunohistochemical marker for human BPDC neoplasm. (Ref.: Montes-Moreno, S., et al. (2013). Blood. 121(4), 643-647).