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Gene Review

Su(H)  -  Suppressor of Hairless

Drosophila melanogaster

Synonyms: BG:DS00929.10, CBF1, CG3497, CSL, Dmel\CG3497, ...
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Disease relevance of Su(H)


High impact information on Su(H)

  • Suppressor of Hairless [Su(H)]/Lag-1/RBP-Jkappa/CBF1 is the only known transducing transcription factor for Notch receptor signaling [2].
  • Upon ligand activation, the intracellular domain of Notch (ICN) translocates to the nucleus, and interacts directly with the DNA-binding protein Suppressor of hairless (Su(H)) in flies, or recombination signal binding protein Jkappa (RBP-Jkappa) in mammals, to activate gene transcription [3].
  • Using many deficiency mutants and in situ hybridization, we mapped the Drosophila J kappa RBP gene in a region containing two recessive lethal mutations, i.e., br26 and br7, which shows the dominant Suppressor of Hairless (Su(H)) phenotype in heterozygotes [4].
  • Proneural enhancement by Notch overcomes Suppressor-of-Hairless repressor function in the developing Drosophila eye [5].
  • The Drosophila RBP-J kappa protein expressed in COS cells bound to the J kappa recognition sequence with the same specificity as the murine counterpart [6].

Biological context of Su(H)


Analytical, diagnostic and therapeutic context of Su(H)


  1. Epstein-Barr virus nuclear antigen 2 exerts its transactivating function through interaction with recombination signal binding protein RBP-J kappa, the homologue of Drosophila Suppressor of Hairless. Zimber-Strobl, U., Strobl, L.J., Meitinger, C., Hinrichs, R., Sakai, T., Furukawa, T., Honjo, T., Bornkamm, G.W. EMBO J. (1994) [Pubmed]
  2. A notch-independent activity of suppressor of hairless is required for normal mechanoreceptor physiology. Barolo, S., Walker, R.G., Polyanovsky, A.D., Freschi, G., Keil, T., Posakony, J.W. Cell (2000) [Pubmed]
  3. MAML1, a human homologue of Drosophila mastermind, is a transcriptional co-activator for NOTCH receptors. Wu, L., Aster, J.C., Blacklow, S.C., Lake, R., Artavanis-Tsakonas, S., Griffin, J.D. Nat. Genet. (2000) [Pubmed]
  4. The Drosophila homolog of the immunoglobulin recombination signal-binding protein regulates peripheral nervous system development. Furukawa, T., Maruyama, S., Kawaichi, M., Honjo, T. Cell (1992) [Pubmed]
  5. Proneural enhancement by Notch overcomes Suppressor-of-Hairless repressor function in the developing Drosophila eye. Li, Y., Baker, N.E. Curr. Biol. (2001) [Pubmed]
  6. The Drosophila RBP-J kappa gene encodes the binding protein for the immunoglobulin J kappa recombination signal sequence. Furukawa, T., Kawaichi, M., Matsunami, N., Ryo, H., Nishida, Y., Honjo, T. J. Biol. Chem. (1991) [Pubmed]
  7. Recognition sequence of a highly conserved DNA binding protein RBP-J kappa. Tun, T., Hamaguchi, Y., Matsunami, N., Furukawa, T., Honjo, T., Kawaichi, M. Nucleic Acids Res. (1994) [Pubmed]
  8. The genetics of a small autosomal region of Drosophila melanogaster containing the structural gene for alcohol dehydrogenase. III. Hypomorphic and hypermorphic mutations affecting the expression of hairless. Ashburner, M. Genetics (1982) [Pubmed]
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