Human SBDS antibody

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.

Product Specifications

Product Name Alternative

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

Host

Mouse

Antigen Species

Human

Reactivity

Human

Immunogen

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

Clonality

Monoclonal

Isotype

IgG2b κ

Clone

AT1E8

Conjugation

Unconjugated

Applications

ELISA, WB, ICC/IF

Purification Method

By protein-G affinity chromatography

Concentration

1 mg/mL (determined by BCA assay)

Additionnal Information

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

References & Citations

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.

Storage Conditions

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.

Formulation

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

Applications Notes

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.

Scientific Category

Metabolism

NCBI Accession Number

NP_057122

Uniprot Accession Number

Q9Y3A5

WB Description

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

IF Description

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) .