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Product Name Anti-SBDS/APC Chinese Name APC标记的SBDS蛋白抗体 Alias Shwachman Bodian-Diamond syndrome; 4733401P19Rik; AI836084; CGI 97; CGI-97; FLJ10917; MGC105922; Protein 22A3; Ribosome maturation protein SBDS; sbds; SBDS_HUMAN; SDS; Shwachman Bodian Diamond syndrome protein; Shwachman Bodian Diamond syndrome protein homolog; Shwachman-Bodian-Diamond syndrome protein; SWDS Research Area Cell biology Cyclin Epigenetics Immunogen Species Rabbit Clonality Polyclonal React Species (predicted: Human, Mouse, Rat, Pig, Horse, Sheep, ) Applications ICC=1:50-200 IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Molecular weight 29kDa Form Lyophilized or Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human SBDS Lsotype IgG Purification affinity purified by Protein A Storage Buffer 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. Product Detail background:
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]
Function:
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.
Subcellular Location:
Cytoplasm. Nucleus > nucleolus. Nucleus > nucleoplasm. Cytoplasm > cytoskeleton > spindle. Primarily detected in the cytoplasm, and at low levels in nucleus and nucleolus (PubMed:19602484 and PubMed:17475909). Detected in the nucleolus during G1 and G2 phase of the cell cycle, and diffusely distributed in the nucleus during S phase. Detected at the mitotic spindle. Colocalizes with the microtubule organizing center during interphase.
Tissue Specificity:
Widely expressed.
DISEASE:
Defects in SBDS are the cause of Shwachman-Diamond syndrome (SDS) [MIM:260400]. SDS is an autosomal recessive disorder characterized by pancreatic exocrine insufficiency, hematologic dysfunction, and skeletal abnormalities.
Similarity:
Belongs to the SDO1/SBDS family.
Database links:Entrez Gene: 51119 Human
Entrez Gene: 66711 Mouse
Omim: 607444 Human
SwissProt: Q9Y3A5 Human
SwissProt: P70122 Mouse
Unigene: 12631 Cow
Unigene: 110445 Human
Unigene: 280484 Mouse
Unigene: 107123 Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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