Human Sp100 [His], recombinant protein from Baculovirus
Recombinant human Sp100, his-tagged
SDS-PAGE (purity > 80%); Western blot with i: PBC sera; ii: monoclonal anti-hexa-His-tag antibody.
0.5-0.9 μg/ml (depending on the type of ELISA plate and coating buffer). Suitable for labeling of functional groups.
2-8°C short term, -20°C long term
This gene encodes a subnuclear organelle and major component of the PML (promyelocytic leukemia)-SP100 nuclear bodies. PML and SP100 are covalently modified by the SUMO-1 modifier, which is considered crucial to nuclear body interactions. The encoded protein binds heterochromatin proteins and is thought to play a role in tumorigenesis, immunity, and gene regulation. Alternatively spliced variants have been identified for this gene; one of which encodes a high-mobility group protein. [provided by RefSeq, Aug 2011]
Primary biliary cirrhosis (PBC) is an autoimmune chronic cholestatic liver disease characterized by destruction of intrahepatic bile ducts and development of cirrhosis and liver failure. Serologically, PBC is characterized by the presence of antimitochondrial (AMA, M2) antibodies. The immunological profil of PBC also includes antinuclear antibodies (ANA), presenting two distinct patterns, multiple nuclear dots (MND) and rim-like/membranous patterns in IIF. Sp100 and promyelocytic leukemia antigen account for the MND pattern, and gp210, nucleoporin p62 and lamin B receptor are associated with nuclear membrane staining
SP100; SP100 nuclear antigen; lysp100b; nuclear autoantigen Sp-100; speckled 100 kDa; SP100-HMG nuclear autoantigen; nuclear dot-associated Sp100 protein;