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Human SBDS antibody

CAT: 0112-ATGA0231-100Size: 100 µLDry Ice: NoHazardous: No
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CAT#:0112-ATGA0231-100Size:100 µL
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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.
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) .

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