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Rabbit Anti-CBL2/RBITC Conjugated antibody (bs-2757R-RBITC)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-2757R-RBITC
英文名稱1 Rabbit Anti-CBL2/RBITC Conjugated antibody
中文名稱 羅丹明(RBITC)標記的原癌蛋白CBL2抗體
別    名 C CBL; Cas-Br-M (murine) ecotropic retroviral transforming sequence; Casitas B lineage lymphoma proto oncogene; CBL 2;E3 ubiquitin protein ligase CBL; Oncogene CBL2; Proto oncogene c CBL; RGD1561386; RING finger protein 55; RNF55v Signal transduction protein CBL; 4732447J05Rik; CBL_HUMAN.  
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  細胞生物  染色質和核信號  神經(jīng)生物學  信號轉導  細胞凋亡  生長因子和激素  轉錄調(diào)節(jié)因子  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, )
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 100kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human CBL2
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. The phosphorylated C-terminus interacts with CD2AP via its second SH3 domain. Binds to UBE2L3. Interacts with adapters SLA, SLA2 and with the phosphorylated C-terminus of SH2B2. Interacts with EGFR, SYK and ZAP70 via the highly conserved Cbl-N region. Also interacts with SORBS1 and INPPL1/SHIP2. Interacts with phosphorylated LAT2. May interact with CBLB (By similarity). Interacts with ALK, AXL, BLK, FGR and FGFR2. Interacts with CSF1R, EPHB1, FLT1, KDR, PDGFRA and PDGFRB; regulates receptor degradation through ubiquitination. Interacts with HCK and LYN. Interacts with TEK/TIE2 (tyrosine phosphorylated).

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

Post-translational modifications:
Phosphorylated on tyrosine residues by ALK, EGFR, SYK, FYN and ZAP70 (By similarity). Phosphorylated on tyrosine residues in response to FLT1 and KIT signaling. Phosphorylated on tyrosine residues by INSR and FGR. Phosphorylated on several tyrosine residues by constitutively activated FGFR3. Not phosphorylated at Tyr-731 by FGFR3. Phosphorylated on tyrosine residues by activated CSF1R, PDGFRA and PDGFRB. Phosphorylated on tyrosine residues by HCK.
Ubiquitinated, leading to its degradation via the proteasome.

DISEASE:
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. Some have an increased risk for certain malignancies, particularly juvenile myelomonocytic leukemia. Note=The disease is caused by mutations affecting the gene represented in this entry.

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.

????CBL2(Casitas B-lineage lymphoma)是一類廣泛分布的細胞內(nèi)蛋白,屬于泛素連接酶E3, Cbl參與細胞內(nèi)信號轉導的負向調(diào)控,這對于維持細胞的內(nèi)穩(wěn)態(tài)具有重要作用。Cbl突變后可成為癌蛋白;許多腫瘤細胞則表現(xiàn)為與增殖有關的分子(如受體型蛋白酪氨酸激酶,RTK)因發(fā)生突變或其它遺傳學改變不受Cbl負調(diào)控。加強或重建Cbl的負調(diào)控作用,或許能夠從受體活化的上游信號開始抑制腫瘤細胞增殖。為利用Cbl的泛素連接酶活性,并使其能夠特異性地降解某些信號轉導分子,從而對一些與腫瘤生長有關的細胞增殖進行負向調(diào)節(jié).
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