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Rabbit Anti-CBL2/BF350 Conjugated antibody (bs-5147R-BF350)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-5147R-BF350
英文名稱 Rabbit Anti-CBL2/BF350 Conjugated antibody
中文名稱 BF350標記的原癌基因CBL2抗體
別    名 C CBL; CBL C; Cas Br M (murine) ecotropic retroviral transforming sequence; Cas-Br-M (murine) ecotropic retroviral transforming sequence; Casitas B lineage lymphoma proto oncogene; Casitas B-lineage lymphoma proto-oncogene; CBL 2; cbl; CBL_HUMAN; CBL2; E3 ubiquitin protein ligase CBL; E3 ubiquitin-protein ligase CBL; Oncogene CBL2; Proto oncogene c CBL; Proto-oncogene c-CBL; RGD1561386; RING finger protein 55; RNF55; Signal transduction protein CBL.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 腫瘤  免疫學  染色質(zhì)和核信號  信號轉導  細胞凋亡  生長因子和激素  轉錄調(diào)節(jié)因子  細胞膜受體  b-淋巴細胞  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, )
產(chǎn)品應用 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 99kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human C CBL
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 is a proto-oncogene that encodes a RING finger E3 ubiquitin ligase. The encoded protein is one of the enzymes required for targeting substrates for degradation by the proteasome. This protein mediates the transfer of ubiquitin from ubiquitin conjugating enzymes (E2) to specific substrates. This protein also contains an N-terminal phosphotyrosine binding domain that allows it to interact with numerous tyrosine-phosphorylated substrates and target them for proteasome degradation. As such it functions as a negative regulator of many signal transduction pathways. This gene has been found to be mutated or translocated in many cancers including acute myeloid leukaemia. Mutations in this gene are also the cause of Noonan syndrome-like disorder. [provided by RefSeq, Mar 2012]

Function:
Adapter protein that functions as a negative regulator of many signaling pathways that are triggered by activation of cell surface receptors. Acts as an E3 ubiquitin-protein ligase, which accepts ubiquitin from specific E2 ubiquitin-conjugating enzymes, and then transfers it to substrates promoting their degradation by the proteasome. Recognizes activated receptor tyrosine kinases, including KIT, FLT1, FGFR1, FGFR2, PDGFRA, PDGFRB, EGFR, CSF1R, EPHA8 and KDR and terminates signaling. Recognizes membrane-bound HCK and other kinases of the SRC family and mediates their ubiquitination and degradation. Participates in signal transduction in hematopoietic cells. Plays an important role in the regulation of osteoblast differentiation and apoptosis. Essential for osteoclastic bone resorption. The Tyr-731 phosphorylated form induces the activation and recruitment of phosphatidylinositol 3-kinase to the cell membrane in a signaling pathway that is critical for osteoclast function.

Subunit:
Interacts (phosphorylated at Tyr-731) with PIK3R1. Associates with NCK via its SH3 domain.

Subcellular Location:
Cytoplasm. Cell membrane. Note=Colocalizes with FGFR2 in lipid rafts at the cell membrane.

DISEASE:
Defects in CBL are the cause of Noonan syndrome-like disorder with or without juvenile myelomonocytic leukemia (NSLL) [MIM:613563]. A syndrome characterized by a phenotype reminiscent of Noonan syndrome. Clinical features are highly variable, including facial dysmorphism, short neck, developmental delay, hyperextensible joints and thorax abnormalities with widely spaced nipples. The facial features consist of triangular face with hypertelorism, large low-set ears, ptosis, and flat nasal bridge. Some patients manifest cardiac defects.

Similarity:
Contains 1 Cbl-PTB (Cbl-type phosphotyrosine-binding) domain.
Contains 1 RING-type zinc finger.
Contains 1 UBA domain.

Database links:

Entrez Gene: 867 Human

Entrez Gene: 12402 Mouse

Entrez Gene: 500985 Rat

Omim: 165360 Human

SwissProt: P22681 Human

SwissProt: P22682 Mouse

Unigene: 504096 Human

Unigene: 266871 Mouse



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

C CBL是泛素一蛋白酶體(ubiquitin proteasome)通路中的一個新的RINGFinger型泛素連接酶(ubiquitinligase ,E3).C CBL可以介導受體酪氨酸激酶和非受體酪氨酸受體激酶的降解.
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