SBDS (Shwachman-Bodian-Diamond Syndrome)

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£406.00

SKU:: 137200
Reinheit:: Purified by Protein G affinity chromatography.
Lagerbedingungen:: -20°C
Hersteller:: United States Biological
Host:: Mouse
Immunogen:: Recombinant protein corresponding to human SBDS, expressed in E.coli.
Klon:: 16C88
Format:: Supplied as a liquid in PBS, pH 7.4, 0.09% sodium azide.
Weitere Details:: The Shwachman-Bodian-Diamond syndrome (SBDS) is 249aa 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. Applications: Suitable for use in ELISA, Western Blot. Other applications not tested. Recommended Dilution: Western Blot: 1:3000 Optimal dilutions to be determined by the researcher. Storage and Stability: May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.