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Rabbit Anti-SBDS/AP Conjugated antibody (bs-20311R-AP)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號 bs-20311R-AP
英文名稱1 Rabbit Anti-SBDS/AP Conjugated antibody
中文名稱 堿性磷酸酶(AP)標(biāo)記的SBDS蛋白抗體
別    名 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  
規(guī)格價(jià)格 100ul/2980元 購買        大包裝/詢價(jià)
說 明 書 100ul  
研究領(lǐng)域 細(xì)胞生物  細(xì)胞周期蛋白  表觀遺傳學(xué)  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, Pig, Horse, Sheep, )
產(chǎn)品應(yīng)用 IHC-P=1:50-200 IHC-F=1:50-200 ICC=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 29kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human SBDS
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 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.
產(chǎn)品介紹 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: 513237 Cow

Entrez Gene: 66711 Mouse

Entrez Gene: 288615 Rat

Omim: 607444 Human

SwissProt: Q3SWZ6 Cow

SwissProt: Q9Y3A5 Human

SwissProt: P70122 Mouse

SwissProt: Q5RK30 Rat

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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