Product Name
Shwachman-Bodian-Diamond Syndrome (SBDS), Monoclonal Antibody
Popular Item
Full Product Name
Mouse Anti Human Shwachman-Bodian-Diamond Syndrome
Product Synonym Names
SBDS Antibody; Shwachman-Bodian-Diamond Syndrome, Mouse Anti Human; SDS; SWDS; Shwachman-Bodian-Diamond syndrome; Ribosome Maturation protein SBDS
Product Gene Name
anti-SBDS antibody
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Isotype
Mouse IgG2b heavy chain and kappa light chain
Purity/Purification
SBDS antibody was purified from mouse ascitic fluids by protein-G affinity chromatography
Form/Format
1mg/ml containing PBS, pH-7.4, 0.02% sodium azide and 10% Glycerol
Immunogen
Anti-human SBDS mAb, is derived from hybridization of mouse FO myeloma cells with spleen cells from BALB/c mice immunized with recombinant human SBDS amino acids 1-250 purified from E Coli.
Physical Appearance
Sterile filtered colorless solution
Preparation and Storage
For periods up to 1 month store at 4°C, for longer periods of time, store at -20°C.
Prevent freeze thaw cycles.
12 months at -20°C. 1 month at 4°C
Other Notes
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.
Related Product Information for
anti-SBDS antibody
SBDS is part of an extremely preserved protein family which exists from archaea to vertebrates and plants. SBDS protein functions in RNA metabolism and has a role in the biogenesis of the 60S ribosomal subunit and translational activation of ribosomes. Shwachman-Diamond syndrome is a rare autosomal recessive disorder produced by mutations in the SBDS gene.
Product Categories/Family for anti-SBDS antibody
MONOCLONAL/POLYCLONAL ANTIBODIES, MISCELLANEOUS ANTIBODIES; Monoclonal Antibodies
NCBI/Uniprot data below describe general gene information for SBDS. It may not necessarily be applicable to this product.
NCBI Accession #
AAH65700.1
[Other Products]
UniProt Secondary Accession #
Q96FX0; Q9NV53; A8K0P4[Other Products]
UniProt Related Accession #
Q9Y3A5[Other Products]
NCBI Official Full Name
Shwachman-Bodian-Diamond syndrome
NCBI Official Synonym Full Names
Shwachman-Bodian-Diamond syndrome
NCBI Official Symbol
SBDS??[Similar Products]
NCBI Official Synonym Symbols
SDS; SWDS; CGI-97
??[Similar Products]
NCBI Protein Information
ribosome maturation protein SBDS
UniProt Protein Name
Ribosome maturation protein SBDS
UniProt Synonym Protein Names
Shwachman-Bodian-Diamond syndrome protein
Protein Family
Ribosome maturation protein
UniProt Gene Name
SBDS??[Similar Products]
UniProt Entry Name
SBDS_HUMAN
NCBI Summary for SBDS
This gene encodes a member of a highly conserved protein family that exists from archaea to vertebrates and plants. The encoded protein may function in RNA metabolism. Mutations within this gene are associated with Shwachman-Bodian-Diamond syndrome. An alternative transcript has been described, but its
biological nature has not been determined. This gene has a closely linked pseudogene that is distally located. [provided by RefSeq, Jul 2008]
UniProt Comments for SBDS
SBDS: Required for the assembly of mature ribosomes and ribosome biogenesis. Together with EFTUD1, 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. Defects in SBDS are the cause of Shwachman-Diamond syndrome (SDS). SDS is an autosomal recessive disorder characterized by pancreatic exocrine insufficiency, hematologic dysfunction, and skeletal abnormalities. Belongs to the SDO1/SBDS family.
Protein type: Nucleolus
Chromosomal Location of Human Ortholog: 7q11.21
Cellular Component: nucleoplasm; spindle pole; cytoplasm; nucleolus; nucleus
Molecular Function: rRNA binding; protein binding; microtubule binding; ribosome binding
Biological Process: bone marrow development; cell proliferation; leukocyte chemotaxis; ribosomal large subunit biogenesis and assembly; inner cell mass cell proliferation; mitotic spindle stabilization; bone mineralization; mature ribosome assembly; rRNA processing
Disease: Shwachman-diamond Syndrome; Aplastic Anemia
Research Articles on SBDS
1. The interaction between EFL1 and SBDS was analyzed by size exclusion chromatography, gel shift assay, and isothermal titration calorimetry. The results showed that EFL1 interacted directly with SBDS.
Precautions
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