货品编号： Bioss.bs-0747R

品牌：Bioss

品名：抗体RANKL

规格：50ul

产品介绍 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.