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

For Research Use Only. Not for use in diagnostic procedures.

Monoclonal

IgG2b,k

AT1E8

Mouse

By protein-G affinity chromatography

Liquid. In phosphate-buffered saline (pH 7.4) with 0.02% Sodium Azide, 10% glycerol.

1mg/ml (lot specific)

Can be stored at 4 degree C.
For long term storage, aliquot and store at -20 degree C.
Avoid repeated freezing and thawing cycles.

Small volumes of anti-SBDS antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

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.

Metabolism

ELISA (EIA), Western Blot (WB), Immunocytochemistry (ICC), Immunofluorescence (IF)

WB: 1:3000.

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 line, stained with DAPI (Blue) for nucleus staining and monoclonal anti-human SBDS antibody (1:100) with goat anti-mouse IgG-Alexa fluor 488 conjugate (Green).

28,764 Da

SBDS, ribosome maturation factor

ribosome maturation protein SBDS

Ribosome maturation protein SBDS

This gene encodes a highly conserved protein that plays an essential role in ribosome biogenesis. The encoded protein interacts with elongation factor-like GTPase 1 to disassociate eukaryotic initiation factor 6 from the late cytoplasmic pre-60S ribosomal subunit allowing assembly of the 80S subunit. Mutations within this gene are associated with the autosomal recessive disorder Shwachman-Bodian-Diamond syndrome. This gene has a closely linked pseudogene that is distally located. [provided by RefSeq, Jan 2017]

Required for the assembly of mature ribosomes and ribosome biogenesis. Together with EFL1, triggers the GTP-dependent release of EIF6 from 60S pre-ribosomes in the cytoplasm, thereby activating ribosomes for translation competence by allowing 80S ribosome assembly and facilitating EIF6 recycling to the nucleus, where it is required for 60S rRNA processing and nuclear export. Required for normal levels of protein synthesis. May play a role in cellular stress resistance. May play a role in cellular response to DNA damage. May play a role in cell proliferation.

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

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