英文名稱 BLNK
中文名稱 B淋巴細(xì)胞連接蛋白抗體
別    名 B cell adapter containing SH2 domain protein; B cell adapter containing Src homology 2 domain protein; B cell linker; B cell linker protein; B cell linker protein; B-cell adapter containing a SH2 domain protein; B-cell adapter containing a Src homology 2 domain protein; B-cell linker protein; BASH; BASH; Bca; Bca; BLNK; BLNK s; BLNK_HUMAN; Cytoplasmic adapter protein; Ly 57; Ly-57; Ly57; Lymphocyte antigen 57; Lymphocyte antigen-57; Lyw 57; Lyw-57; Lyw57; MGC111051; SLP 65; SLP65; SLP-65; Src homology 2 domain containing leukocyte protein of 65 kDa.
B淋巴細(xì)胞連接蛋白抗體說 明 書 0.1ml  0.2ml  
研究領(lǐng)域 免疫學(xué)  信號轉(zhuǎn)導(dǎo)  細(xì)胞粘附分子  細(xì)胞表面分子  b-淋巴細(xì)胞  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, 
產(chǎn)品應(yīng)用 WB=1:100-500 ELISA=1:500-1000 IP=1:20-100 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蠟切片需做抗原修復(fù)）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 65kDa
細(xì)胞定位 細(xì)胞漿 細(xì)胞膜 
性    狀 Lyophilized or Liquid
濃    度 1mg/1ml
免 疫 原 KLH conjugated synthetic peptide derived from human BLNK
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M PBS, pH 7.4 with 10 mg/ml BSA and 0.1% Sodium azide
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 cytoplasmic linker or adaptor protein that plays a critical role in B cell development. This protein bridges B cell receptor-associated kinase activation with downstream signaling pathways, thereby affecting various biological functions. The phosphorylation of five tyrosine residues is necessary for this protein to nucleate distinct signaling effectors following B cell receptor activation. Mutations in this gene cause hypoglobulinemia and absent B cells, a disease in which the pro- to pre-B-cell transition is developmentally blocked. Deficiency in this protein has also been shown in some cases of pre-B acute lymphoblastic leukemia. Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, May 2012].

Function:
Functions as a central linker protein that bridges kinases associated with the B-cell receptor (BCR) with a multitude of signaling pathways, regulating biological outcomes of B-cell function and development. Plays a role in the activation of ERK/EPHB2, MAP kinase p38 and JNK. Modulates AP1 activation. Important for the activation of NF-kappa-B and NFAT. Plays an important role in BCR-mediated PLCG1 and PLCG2 activation and Ca(2+) mobilization and is required for trafficking of the BCR to late endosomes. However, does not seem to be required for pre-BCR-mediated activation of MAP kinase and phosphatidyl-inositol 3 (PI3) kinase signaling. May be required for the RAC1-JNK pathway. Plays a critical role in orchestrating the pro-B cell to pre-B cell transition (By similarity). Plays an important role in BCR-induced B-cell apoptosis.

Subunit:
Associates with PLCG1, VAV1 and NCK1 in a B-cell antigen receptor-dependent fashion. Interacts with VAV3, PLCG2 and GRB2. Interacts through its SH2 domain with CD79A.

Subcellular Location:
Cytoplasm. Cell membrane. BCR activation results in the translocation to membrane fraction.

Tissue Specificity:
Expressed in B-cell lineage and fibroblast cell lines (at protein level). Highest levels of expression in the spleen, with lower levels in the liver, kidney, pancreas, small intestines and colon.

Post-translational modifications:
Following BCR activation, phosphorylated on tyrosine residues by SYK and LYN. When phosphorylated, serves as a scaffold to assemble downstream targets of antigen activation, including PLCG1, VAV1, GRB2 and NCK1. Phosphorylation of Tyr-84, Tyr-178 and Tyr-189 facilitates PLCG1 binding. Phosphorylation of Tyr-96 facilitates BTK binding. Phosphorylation of Tyr-72 facilitates VAV1 and NCK1 binding. Phosphorylation is required for both Ca(2+) and MAPK signaling pathways.

DISEASE:
Defects in BLNK are the cause of agammaglobulinemia type 4 (AGM4) [MIM:613502]. It is a primary immunodeficiency characterized by profoundly low or absent serum antibodies and low or absent circulating B cells due to an early block of B-cell development. Affected individuals develop severe infections in the first years of life.

Similarity:
Contains 1 SH2 domain.

Gene ID:
29760

Database links:

Entrez Gene: 29760 Human

Entrez Gene: 17060 Mouse

Entrez Gene: 499356 Rat

Omim: 604515 Human

SwissProt: Q8WV28 Human

SwissProt: Q9QUN3 Mouse

SwissProt: Q4KM52 Rat

Unigene: 665244 Human

Unigene: 9749 Mouse

Unigene: 32684 Rat

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