Alternative titles; symbols
HGNC Approved Gene Symbol: ELOB
Cytogenetic location: 16p13.3 Genomic coordinates (GRCh38): 16:2,771,414-2,777,280 (from NCBI)
The elongin (SIII) complex activates elongation by mammalian RNA polymerase II by suppressing transient pausing of the polymerase at many sites within transcription units. Elongin is a heterotrimer composed of A (ELOA; 600786), B, and C (600788) subunits of 110, 18, and 15 kilodaltons, respectively (summary by Aso et al., 1995).
Aso et al. (1995) demonstrated that elongin A functions as the transcriptionally active component of the SIII complex, whereas elongins B and C are regulatory subunits.
Duan et al. (1995) and Kibel et al. (1995) reported results suggesting that the tumor suppression activity of the von Hippel-Lindau (VHL; 608537) tumor suppressor gene product is a function of its ability to bind to elongins B and C and thereby inhibit transcription elongation. A preponderance of the tumor-disposing inherited missense mutations detected in von Hippel-Lindau disease are within the elongin-binding domain of VHL. Schoenfeld et al. (2000) reported that VHL proteins harboring mutations that disrupt elongin binding were unstable and rapidly degraded by the proteasome. In contrast, wildtype VHL proteins were directly stabilized by associating with both elongins B and C. In addition, elongins B and C were stabilized through their interactions with each other and VHL. Thus, the entire VHL/elongin complex is resistant to proteasomal degradation. Because the elongin-binding domain of VHL is frequently mutated in cancers, these results suggest that loss of elongin binding causes tumorigenesis by compromising VHL protein stability and/or potential VHL ubiquitination functions.
Aso, T., Lane, W. S., Conaway, J. W., Conaway, R. C. Elongin (SIII): a multisubunit regulator of elongation by RNA polymerase II. Science 269: 1439-1443, 1995. [PubMed: 7660129] [Full Text: https://doi.org/10.1126/science.7660129]
Duan, D. R., Pause, A., Burgess, W. H., Aso, T., Chen, D. Y. T., Garrett, K. P., Conaway, R. C., Conaway, J. W., Linehan, W. M., Klausner, R. D. Inhibition of transcription elongation by the VHL tumor suppressor protein. Science 269: 1402-1406, 1995. [PubMed: 7660122] [Full Text: https://doi.org/10.1126/science.7660122]
Kibel, A., Iliopoulos, O., DeCaprio, J. A., Kaelin, W. G., Jr. Binding of the von Hippel-Lindau tumor suppressor protein to elongin B and C. Science 269: 1444-1446, 1995. [PubMed: 7660130] [Full Text: https://doi.org/10.1126/science.7660130]
Schoenfeld, A. R., Davidowitz, E. J., Burk, R. D. Elongin BC complex prevents degradation of von Hippel-Lindau tumor suppressor gene products. Proc. Nat. Acad. Sci. 97: 8507-8512, 2000. [PubMed: 10900011] [Full Text: https://doi.org/10.1073/pnas.97.15.8507]