Catalogue number: | PA1800 |
Price: | $200.00 |
Reactivities: | Human, Mouse, Rat |
Applications: | Western Blot |
Size: | 100ug/vial |
Gene: | CBL |
Swiss prot: | P22681 |
Form: | Lyophilized |
Format: | Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg Thimerosal, 0.05mg NaN3. |
Storage temp: | At -20 degree C for one year. After reconstitution, at 4 degree C for one month. It can also be aliquotted and stored frozen at -20 degree C for a longer time.Avoid repeated freezing and thawing. |
Scientific background: | 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. |
References: | 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. |
Additional info: | A synthetic peptide corresponding to a sequence at the C-terminal of human CBL, identical to the related rat and mouse sequences. |