Anti-CBL Antibody Picoband® Fluoro488 Conjugated

CBL (Cbl proto-oncogene) is also known as C-CBL, RNF55, CBL2 and E3 ubiquitin protein ligase. Wei et al. (1990) mapped the CBL gene to chromosome 11q23.3-qter by molecular characterization of the breakpoints in 2 somatic cell hybrids. 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.

E3 ubiquitin-protein ligase CBL;6.3.2.-; Casitas B-lineage lymphoma proto-oncogene; Proto-oncogene c-Cbl; RING finger protein 55; Signal transduction protein CBL; CBL; CBL2, RNF55

Adapters, Cancer, Cancer Susceptibility, Cell Biology, Co-Activators/Co-Repressors, Cytoplasmic, Epigenetics and Nuclear Signaling, Nuclear Hormone Receptors, Nuclear Receptors, Nuclear Signaling, Nuclear Signaling Pathways, Oncoproteins, Oncoproteins/Suppressors, Proteasome / Ubiquitin, Proteolysis/Ubiquitin, Proto-Oncogenes, Ring Finger E3 Ligase, Signal Transduction, Signaling Pathway, Transcription, Ubiquitin E3 Enzymes

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, SRC 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. May be functionally coupled with the E2 ubiquitin-protein ligase UB2D3. .

1. Joazeiro, C. A. P., Wing, S. S., Huang, H., Leverson, J. D., Hunter, T., Liu, Y.-C. The tyrosine kinase negative regulator c-Cbl as a RING-type, E2-dependent ubiquitin-protein ligase. Science 286: 309-312, 1999. 2. Martinelli, S., De Luca, A., Stellacci, E., Rossi, C., Checquolo, S., Lepri, F., Caputo, V., Silvano, M., Buscherini, F., Consoli, F., Ferrara, G., Digilio, M. C., and 14 others.Heterozygous germline mutations in the CBL tumor-suppressor gene cause a Noonan syndrome-like phenotype. Am. J. Hum. Genet. 87: 250-257, 2010. 3. Wei, S., Rocchi, M., Archidiacono, N., Sacchi, N., Romeo, G., Gatti, R. A. Physical mapping of the human chromosome 11q23 region containing the ataxia-telangiectasia locus. Cancer Genet. Cytogenet. 46: 1-8, 1990.