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Description

Nuclear autoantigen Sp-100 (UniProt P23497, also known as Nuclear dot-associated Sp100 protein, Speckled 100 kDa) is encoded by the SP100 gene (Gene ID 6672) in human. The SP100 family genes, including SP100, SP110, SP140 and SP140L, encode highly similar proteins mainly expressed in leukocytes. Interferons (IFNs) and other viral infection-related stimuli induce SP100 family gene expression and mutations of SP100 genes are linked to immune diseases and cancers. SP100 colocalizes with promyelocytic leukemia (PML) protein in nuclear bodies (NBs), also known as nuclear domain 10 (ND10), that function to regulate gene transcription via chromatin-based mechanisms and thereby mediate a variety of cellular functions including senescence, apoptosis, protein degradation, telemere stasbility, and defense against viral infections.