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Rabbit Anti-RANKL/CD254/BF647 Conjugated antibody (bs-0747R-BF647)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-0747R-BF647
英文名稱 Rabbit Anti-RANKL/CD254/BF647 Conjugated antibody
中文名稱 BF647標記的骨保護蛋白配體/破骨細胞分化因子抗體
別    名 OPGL; CD254; hRANKL2; ODF; OPGL; Osteoclast differentiation factor; Osteoprotegerin ligand; RANKL; Receptor activator of nuclear factor kappa B ligand; sOdf; SOFA; TNF related activation induced cytokine; TNFSF 11; TNFSF11; TRANCE; Tumor necrosis factor ligand superfamily member 11; Osteoprotegerin Ligand; TNF11_HUMAN.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 腫瘤  細胞生物  免疫學  發育生物學  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human, Mouse,  (predicted: Rat, Dog, )
產品應用 Flow-Cyt=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 35kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human OPGL
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.
產品介紹 background:
This gene encodes a member of the tumor necrosis factor (TNF) cytokine family which is a ligand for osteoprotegerin and functions as a key factor for osteoclast differentiation and activation. This protein was shown to be a dentritic cell survival factor and is involved in the regulation of T cell-dependent immune response. T cell activation was reported to induce expression of this gene and lead to an increase of osteoclastogenesis and bone loss. This protein was shown to activate antiapoptotic kinase AKT/PKB through a signaling complex involving SRC kinase and tumor necrosis factor receptor-associated factor (TRAF) 6, which indicated this protein may have a role in the regulation of cell apoptosis. Targeted disruption of the related gene in mice led to severe osteopetrosis and a lack of osteoclasts. The deficient mice exhibited defects in early differentiation of T and B lymphocytes, and failed to form lobulo-alveolar mammary structures during pregnancy. Two alternatively spliced transcript variants have been found. [provided by RefSeq, Jul 2008].

Function:
Cytokine that binds to TNFRSF11B/OPG and to TNFRSF11A/RANK. Osteoclast differentiation and activation factor. Augments the ability of dendritic cells to stimulate naive T-cell proliferation. May be an important regulator of interactions between T-cells and dendritic cells and may play a role in the regulation of the T-cell-dependent immune response. May also play an important role in enhanced bone-resorption in humoral hypercalcemia of malignancy.

Subcellular Location:
Cytoplasm; Secreted and Cell membrane.

Tissue Specificity:
Highest in the peripheral lymph nodes, weak in spleen, peripheral blood Leukocytes, bone marrow, heart, placenta, skeletal muscle, stomach and thyroid.

Post-translational modifications:
The soluble form of isoform 1 derives from the membrane form by proteolytic processing. The cleavage may be catalyzed by ADAM17.

DISEASE:
Defects in TNFSF11 are the cause of osteopetrosis autosomal recessive type 2 (OPTB2) [MIM:259710]; also known as osteoclast-poor osteopetrosis. Osteopetrosis is a rare genetic disease characterized by abnormally dense bone, due to defective resorption of immature bone. The disorder occurs in two forms: a severe autosomal recessive form occurring in utero, infancy, or childhood, and a benign autosomal dominant form occurring in adolescence or adulthood. Autosomal recessive osteopetrosis is usually associated with normal or elevated amount of non-functional osteoclasts. OPTB2 is characterized by paucity of osteoclasts, suggesting a molecular defect in osteoclast development.

Similarity:
Belongs to the tumor necrosis factor family.

Database links:

Entrez Gene: 8600 Human

Entrez Gene: 21943 Mouse

Omim: 602642 Human

SwissProt: O14788 Human

SwissProt: O35235 Mouse

Unigene: 333791 Human

Unigene: 249221 Mouse



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

OPGL骨保護蛋白配體又稱骨保護素配體(破骨細胞發育刺激因子)。屬腫瘤壞死因子TNF-a家族。
OPGL促進破骨細胞的分化和活性,而OPG抑制這些過程。骨髓瘤細胞影響骨髓中這兩種蛋白的生理平衡,是發生溶骨性病變的根本所在。
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