免疫原
Synthesized peptide derived from human SP110 AA range: 453-503
特异性
This antibody detects endogenous levels of SP110 at Human/Mouse/Rat
组成(Formulation)
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
纯化工艺(Immunogen)
The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen.
背景(Background)
The nuclear body is a multiprotein complex that may have a role in the regulation of gene transcription. This gene is a member of the SP100/SP140 family of nuclear body proteins and encodes a leukocyte-specific nuclear body component. The protein can function as an activator of gene transcription and may serve as a nuclear hormone receptor coactivator. In addition, it has been suggested that the protein may play a role in ribosome biogenesis and in the induction of myeloid cell differentiation. Alternative splicing has been observed for this gene and three transcript variants, encoding distinct isoforms, have been identified. [provided by RefSeq, Jul 2008],
功能
disease:Defects in SP110 are the cause of hepatic venoocclusive disease with immunodeficiency (VODI) [MIM:235550]. VODI is an autosomal recessive primary immunodeficiency associated with hepatic vascular occlusion and fibrosis. The immunodeficiency is characterized by severe hypogammaglobulinemia, combined T and B cell immunodeficiency, absent lymph node germinal centers, and absent tissue plasma cells.,function:Transcription factor. May be a nuclear hormone receptor coactivator. Enhances transcription of genes with retinoic acid response elements (RARE).,induction:By interferon gamma and by all-trans retinoic acid.,online information:SP110 mutation db,PTM:Phosphorylated (isoform 2).,similarity:Contains 1 bromo domain.,similarity:Contains 1 HSR domain.,similarity:Contains 1 PHD-type zinc finger.,similarity:Contains 1 SAND domain.,subcellular location:Found in the nuclear body.,subunit:Is