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|Product Name||Anti-SBDS (clone AT1E8)|
|Description||The Shwachman-Bodian-Diamond syndrome (SBDS) is 249 amino acid ribosome maturation protein. The precise function of the SBDS protein is not known but it appears to play an important role in actin cytoskeletal function and mitotic spindle stabilization. Also, SBDS is required for the assembly of mature ribosomes and ribosome biogenesis. Mutations in the SBDS gene cause Shwachman-Diamond syndrome (SDS). SDS is an autosomal recessive disorder with clinical features that include pancreatic exocrine insufficiency, haematological dysfunction and skeletal abnormalities.|
|Synonyms||Ribosome maturation protein SBDS, CGI-97, SDS, SWDS|
|Clone||AT1E8. Anti-human SBDS mAb, clone AT1E8, is derived from hybridization of mouse F0 myeloma cells with spleen cells from BALB/c mice immunized with a recombinant human SBDS protein.|
|Immunogen||Recombinant human SBDS (1-250aa) purified from E. coli|
|Form||Purified, in Phosphate-Buffered Saline (pH 7.4) with 0.1% Sodium Azide|
|Applications||Dot blot, ELISA, WB|
|Working Dilutions||Optimal dilution should be performed by user.|
|Storage||Can be stored at +4C. For long term storage, aliquot and store at -20C. Avoid repeated freezing and thawing cycles.|
|References||Boocock G.R., et al. (2003) Nat Genet 33(1): 97€“101Orelio C., et al. (2011) PLoS ONE 6(6): e20727Savchenko., et al. (2005) J Biol Chem 280(19): 19213€“20|
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