Humaan SBDS antilichaam

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.

Ribosome maturation protein SBDS, CGI-97, SDS, SWDS

Human

Human

Recombinant human SBDS (1-250aa) purified from E. coli

AT1E8

Unconjugated

ELISA, WB, ICC/IF

By protein-G affinity chromatography

1 mg/mL (determined by BCA assay)

SBDS, ATGA0231-10 µg, ATGA0231-20 µg, ATGA0231-50 µg, ATGA0231-100 µg, ATGA0231-250 µg, ATGA0231-500 µg, ATGA0231-1 mg, ATGA0231-10, ATGA0231-20, ATGA0231-50, ATGA0231-100, ATGA0231-250, ATGA0231-500, ATGA0231-1

Boocock G.R., et al. (2003) Nat Genet 33 (1) : 97-101.; ; Orelio C., et al. (2011) PLoS ONE 6 (6) : e20727.; ; Savchenko., et al. (2005) J Biol Chem 280 (19) : 19213-20.

Can be stored at 2°C to 8°C for 1 week. For long term storage, aliquot and store at -20C to -80C. Avoid repeated freezing and thawing cycles.

Liquid in. Phosphate-Buffered Saline (pH 7.4) with 0.02% Sodium Azide, 10% glycerol

The antibody has been tested by ELISA, Western blot and ICC/IF analysis to assure specificity and reactivity. Since application varies, however, each investigation should be titrated by the reagent to obtain optimal results.

Metabolism

NP_057122

Q9Y3A5

The cell lysates (40ug) were resolved by SDS-PAGE, transferred to PVDF membrane and probed with anti-human SBDS antibody (1:3000) . Proteins were visualized using a goat anti-mouse secondary antibody conjugated to HRP and an ECL detection system.; ; Lane 1.: 293T cell lysate; ; Lane 2.: HeLa cell lysate

ICC/IF analysis of SBDS in HeLa cells. The cell was stained with ATGA0231 (1:100) . The secondary antibody (green) was used Alexa Fluor 488. DAPI was stained the cell nucleus (blue) .