SBDS Antibody

Code CSB-PA897481LA01HU
Size US$166
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  • IHC image of CSB-PA897481LA01HU diluted at 1:400 and staining in paraffin-embedded human colon cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.

  • Immunofluorescence staining of Hela cells with CSB-PA897481LA01HU at 1:133, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).

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Product Details

Full Product Name
Rabbit anti-Homo sapiens (Human) SBDS Polyclonal antibody
Uniprot No.
Target Names
SBDS
Alternative Names
4733401P19Rik antibody; AI836084 antibody; CGI 97 antibody; CGI-97 antibody; FLJ10917 antibody; MGC105922 antibody; Protein 22A3 antibody; Ribosome maturation protein SBDS antibody; sbds antibody; SBDS_HUMAN antibody; SDS antibody; Shwachman Bodian Diamond syndrome protein antibody; Shwachman Bodian Diamond syndrome protein homolog antibody; Shwachman Bodian-Diamond syndrome antibody; Shwachman-Bodian-Diamond syndrome protein antibody; SWDS antibody
Raised in
Rabbit
Species Reactivity
Human
Immunogen
Recombinant Human Ribosome maturation protein SBDS protein (37-153AA)
Immunogen Species
Homo sapiens (Human)
Conjugate
Non-conjugated

The SBDS Antibody (Product code: CSB-PA897481LA01HU) is Non-conjugated. For SBDS Antibody with conjugates, please check the following table.

Available Conjugates
Conjugate Product Code Product Name Application
HRP CSB-PA897481LB01HU SBDS Antibody, HRP conjugated ELISA
FITC CSB-PA897481LC01HU SBDS Antibody, FITC conjugated
Biotin CSB-PA897481LD01HU SBDS Antibody, Biotin conjugated ELISA
Clonality
Polyclonal
Isotype
IgG
Purification Method
>95%, Protein G purified
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
Form
Liquid
Tested Applications
ELISA, IHC, IF
Recommended Dilution
Application Recommended Dilution
IHC 1:200-1:500
IF 1:50-1:200
Troubleshooting and FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

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Target Background

Function
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.
Gene References into Functions
  1. Structural variation in SBDS gene, with loss of exon 3, in two Shwachman-Diamond patients. PMID: 27519942
  2. SBDS mutation is associated with Shwachman Diamond-Syndrome. PMID: 28509441
  3. SBDS function is specifically required for efficient translation re-initiation into the protein isoforms C/EBPalpha-p30 and C/EBPbeta-LIP, which is controlled by a single cis-regulatory upstream open reading frame (uORF) in the 5' untranslated regions (5' UTRs) of both mRNAs. PMID: 26762974
  4. Association of Elongation Factor-like 1 (EFL1) GTPase to SBDS did not modify the affinity for GTP but dramatically decreased that for GDP by increasing the dissociation rate of the nucleotide. PMID: 25991726
  5. The clinical diagnosis was confirmed by detection of compound heterozygous mutations in SBDS using whole-exome sequencing: a recurrent intronic mutation causing aberrant splicing (c.258+2T>C) and a novel missense variant in a highly conserved codon (c.41A>G, p.Asn14Ser), considered to be damaging for the protein structure by in silico prediction programs PMID: 26866830
  6. Upon EFL1 binding, SBDS is repositioned around helix 69, thus facilitating a conformational switch in EFL1 that displaces eIF6 by competing for an overlapping binding site on the 60S ribosomal subunit. PMID: 26479198
  7. Association of EFL1 to SBDS did not modify the affinity for GTP but dramatically decreased that for GDP by increasing the dissociation rate of the nucleotide. PMID: 25991726
  8. Genetic variations in exon 2 of SBDS gene do not appear to be contributing to aplastic anemia in the north Indian population. PMID: 24636098
  9. 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. PMID: 24406167
  10. SBDS protein acts as a nucleotide exchange factor that stabilizes binding to GTP for human GTPase. PMID: 23831625
  11. Lack of mutation in exon 2 of sbds protein in acute myeloid leukemia suggests this subset is unlikely to have underlying sds PMID: 23189942
  12. We conclude that knockdown of SBDS leads to growth inhibition and defects in ribosome maturation PMID: 22997148
  13. SBDS protein facilitates the release of eIF6, a factor that prevents ribosome joining. PMID: 23115272
  14. Erythropoiesis (in normal stem cells or in cells from Shwachman-Diamond syndrome patients) requires SBDS. Knockdown of SBDS leads to oxidative stress, to increased levels of ROS during erythroid differentiation, and disrupts ribosome biogenesis. PMID: 21963601
  15. the amount of mutated SBDS protein was decreased PMID: 21660439
  16. SBDS full-length protein was localized in both the nucleus and cytoplasm, whereas patient-related truncated SBDS protein isoforms localize predominantly to the nucleus. PMID: 21695142
  17. SBDS-deficiency results in deregulation of reactive oxygen species leading to increased cell death and decreased cell growth in cancer. PMID: 20979173
  18. Mutations of the Shwachman-Bodian-Diamond syndrome gene is not associated with refractory cytopenia. PMID: 19951977
  19. determined the solution structure and backbone dynamics of the SBDS protein and describe its RNA binding site using NMR spectroscopy PMID: 20053358
  20. analysis of SBDS expression and localization at the mitotic spindle in human myeloid progenitors PMID: 19759903
  21. Mutations in SBDS are associated with Shwachman-Diamond syndrome PMID: 12496757
  22. gene conversion mutations in SBDS are common to different ethnic groups, but they are not confined to a limited region of the gene PMID: 14749921
  23. most, but not all, patients classified based on rigorous clinical criteria as having SDS had compound heterozygous mutations of SBDS; presence (or absence) of SBDS mutations may define subgroups of patients with SDS PMID: 15284109
  24. patients with genetically proven SDS a genotype-phenotype relationship in SDS does not exist in clinical and hematologic terms PMID: 15769891
  25. SBDS localization was cell-cycle dependent, with nucleolar localization during G1 and G2 and diffuse nuclear localization during S phase PMID: 15860664
  26. analysis of phenotypic heterogeneity in Shwachman-Diamond syndrome patients carrying identical SBDS mutations PMID: 15942154
  27. findings link Shwachman-Diamond syndrome to other bone marrow failure syndromes with defects in nucleolus-associated processes, including Diamond-Blackfan anemia, cartilage-hair hypoplasia, and dyskeratosis congenita PMID: 16529906
  28. This is the first report of compound heterozygous missense mutations occurring in patients with SDS. Two novel missense mutations (c.362A > C in exon 3, and c.523C > T in exon 4) of the SBDS gene were identified in the patient. PMID: 17046571
  29. A novel missense mutation (79TC) in exon 1 is reported in a girl with spondylometaphysial dysplasia, ecpanding the phenotype beyond Shwachman-Bodian-Diamond syndrome. PMID: 17400792
  30. Mutations in the SBDS gene is associated with acquired aplastic anemia PMID: 17478638
  31. SBDS is found in complexes containing the human Nip7 ortholog. PMID: 17643419
  32. summary of documented SBDS mutations associated with Shwachman-Diamond syndrome PMID: 17916435
  33. genetic analysis of SBDS and SH2D1A in Japanese children with AA PMID: 18024409
  34. Mutations in the SBDS gene may therefore be the fifth identified molecular defect in CVID. PMID: 18190602
  35. SBDS has pro-survival properties. Its inhibition results in accelerated apoptosis through the Fas pathway. PMID: 18268284
  36. findings suggest that Shwachman-Diamond syndrome patients with mutations in the SBDS gene have a characteristic magnetic resonance imaging pattern of fat-replaced pancreas and that SBDS mutations are unlikely in patients without this pattern PMID: 18280855
  37. SBDS loss results in abnormal accumulation of Fas at the plasma membrane, where it sensitizes the cells to stimulation by Fas ligand PMID: 19009351
  38. We confirmed significant overexpression of osteoprotegerin and vascular endothelial growth factor-A by ELISA from supernatants of SBDS-depleted HeLa cells. PMID: 19014892
  39. in all cases the i(7)(q10) carries a double dose of the c.258+2T>C. As the c.258+2T>C mutation still allows the production of some amount of normal protein, this may contribute to the low incidence of MDS/AML in this subset of SDS patients PMID: 19148133
  40. SBDS is a multi-functional protein implicated in cellular stress responses. PMID: 19602484
  41. A novel mutation in a Fijian boy with Shwachman-Diamond syndrome. PMID: 19816210
  42. Mutations in SBDS are associated with Shwachman-Diamond syndrome. PMID: 12496757

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Involvement in disease
Shwachman-Diamond syndrome (SDS)
Subcellular Location
Cytoplasm. Nucleus, nucleolus. Nucleus, nucleoplasm. Cytoplasm, cytoskeleton, spindle.
Protein Families
SDO1/SBDS family
Tissue Specificity
Widely expressed.
Database Links

HGNC: 19440

OMIM: 260400

KEGG: hsa:51119

STRING: 9606.ENSP00000246868

UniGene: Hs.110445

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