CBL2,原癌基因CBL2抗体

产品编号HZ-5147R
英文名称CBL2
中文名称原癌基因CBL2抗体
别 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.
说 明 书0.1ml 0.2ml
研究领域肿瘤 免疫学 染色质和核信号 信号转导 细胞凋亡 生长因子和激素 转录调节因子 细胞膜受体 b-淋巴细胞
抗体来源Rabbit
克隆类型Polyclonal
交叉反应Human, Mouse, Rat,
CBL2,原癌基因CBL2抗体产品应用WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量99kDa
细胞定位细胞浆 细胞膜
性 状Lyophilized or Liquid
浓 度1mg/1ml
免 疫 原KLH conjugated synthetic peptide derived from human C CBL
亚 型IgG
纯化方法affinity purified by Protein A
储 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件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.
CBL2,原癌基因CBL2抗体PubMedPubMed
产品介绍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.

CBL2,原癌基因CBL2抗体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是泛素一蛋白酶体(ubiquitin proteasome)通路中的一个新的RINGFinger型泛素连接酶(ubiquitinligase ,E3).C CBL可以介导受体酪氨酸激酶和非受体酪氨酸受体激酶的降解.