Shwachman Bodian Diamond syndrome (SBDS) (1-250) Mouse Monoclonal Antibody [Clone ID: AT1E8]
CAT#: AM50018PU-S
Shwachman Bodian Diamond syndrome (SBDS) (1-250) mouse monoclonal antibody, clone AT1E8, Purified
Size: 100 ul
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CNY 5,630.00
Specifications
Product Data | |
Clone Name | AT1E8 |
Applications | ELISA, IF, WB |
Recommend Dilution | ELISA. Western blot: 1/3000. Immunocytochemistry / Immunoflourescence. |
Reactivity | Human |
Host | Mouse |
Clonality | Monoclonal |
Immunogen | Recombinant human SBDS (1-250aa) purified from E. coli |
Specificity | This antibody detects recombinant Human SBDS (1-250aa) Other species not tested. |
Formulation | PBS, pH 7.4 containing 0.02% Sodium Azide and 10% Glycerol State: Purified State: Liquid purified Ig fraction |
Concentration | lot specific |
Purification | Protein-G affinity chromatography |
Conjugation | Unconjugated |
Storage Condition | Store undiluted at 2-8°C for up to two weeks or (in aliquots) at -20°C for longer. Avoid repeated freezing and thawing. |
Gene Name | SBDS ribosome assembly guanine nucleotide exchange factor |
Database Link | |
Background | 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. |
Synonyms | Shwachman-Bodian-Diamond syndrome protein |
Reference Data |
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