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 mouse 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
细胞定位
Nucleus . Found in the nuclear body.
组织表达
Highly expressed in peripheral blood leukocytes and spleen. Detected at intermediate levels in thymus, prostate, testis, ovary, small intestine and colon, and at low levels in heart, brain, placenta, lung, liver, skeletal muscle, kidney and pancreas.
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