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Gene Review

snRNP-U1-70K  -  small ribonucleoprotein particle U1...

Drosophila melanogaster

Synonyms: 70K, CG8749, Dm70K, Dmel\CG8749, U1, ...
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Disease relevance of snRNP70K

  • A mutant transgene that lacks the PSI-U1 snRNP-interaction domain restores viability but shows courtship behavior abnormalities and meiosis defects during spermatogenesis, resulting in a complete male sterility phenotype [1].
  • Here we describe for the first time the selection and characterization of recombinant human anti-U1-70K single chain autoantibody fragments (anti-hU1-70K scFv) from autoimmune patient-derived phage display antibody libraries [2].
  • Some contain long repeats of Asp-Arg or Glu-Arg, among them the small nuclear ribonucleo-U1 70K protein, which is an autoantigen in systemic lupus erythematosis [3].

High impact information on snRNP70K

  • By site-directed mutagenesis, we determined that the 70K protein consists of 437 amino acids (52 kd), and found that its aberrant electrophoretic migration is due to a carboxy-terminal charged domain structurally similar to two Drosophila proteins (su(wa) and tra) that may regulate alternative pre-messenger RNA splicing [4].
  • The 5' flanking sequences of three distinct 70K genes were also determined [5].
  • Here we summarize a study of the organization of genes coding for the 70K heat shock-specific protein contained in the two recombinant chromosomal DNA plasmids pG3 and pG5 [6].
  • Here, we report that the conserved U1 snRNP-interacting RNA-binding protein PSI is essential for Drosophila viability [1].
  • Direct interactions between RNA-binding proteins and snRNP particles modulate eukaryotic pre-mRNA processing patterns to control gene expression [1].

Biological context of snRNP70K


Anatomical context of snRNP70K


Associations of snRNP70K with chemical compounds

  • Psoralen-mediated crosslinking experiments suggest that SXL binding to this uridine-rich sequence inhibits recognition of the 5' splice site by U1 snRNP in HeLa nuclear extracts [12].
  • Velocity sedimentation in sucrose gradients was used to partially fractionate the RNPs; analysis of fractions so obtained suggests that, in general, one snRNP contains one snRNA [13].
  • Our results are consistent with a role for the purine region in facilitated snRNP binding to the NRS via SF2/ASF [14].
  • The Drosophila U1-70K protein is required for viability, but its arginine-rich domain is dispensable [15].

Physical interactions of snRNP70K

  • The U1-70K protein is specifically bound to stemloop I of the U1 small nuclear RNA contained in the U1 small nuclear ribonucleoprotein complex (U1 snRNP), which is involved in the splicing of pre-mRNA [2].

Regulatory relationships of snRNP70K

  • Both loss-of-function and gain-of-function mutations in snf define a role for snRNP proteins in regulating Sex-lethal pre-mRNA splicing in Drosophila development [16].

Other interactions of snRNP70K

  • These data show that the processing of specific target transcripts, such as the P-element mRNA, is regulated by a functional PSI-U1 snRNP interaction in Drosophila [17].
  • Its role, however, has remained enigmatic because of questions about whether SNF acts as part of an intact snRNP or a free protein [7].
  • In contrast, increasing the dose of genes encoding either the integral U1 snRNP protein U1-70k, or the integral U2 snRNP protein SF3a(60), has no effect [9].
  • Numerous differences in the relative amounts of snRNP particles and hnRNP proteins on nascent transcripts are also observed [10].
  • Based on these studies we suggest a model in which the snRNP protein, Snf, functions with Sex-lethal to block recognition of the regulated male-specific exon [16].

Analytical, diagnostic and therapeutic context of snRNP70K


  1. The KH-type RNA-binding protein PSI is required for Drosophila viability, male fertility, and cellular mRNA processing. Labourier, E., Blanchette, M., Feiger, J.W., Adams, M.D., Rio, D.C. Genes Dev. (2002) [Pubmed]
  2. Characterization of recombinant human autoantibody fragments directed toward the autoantigenic U1-70K protein. Degen, W.G., Pieffers, M., Welin-Henriksson, E., van den Hoogen, F.H., van Venrooij, W.J., Raats, J.M. Eur. J. Immunol. (2000) [Pubmed]
  3. Polar zippers: their role in human disease. Perutz, M. Protein Sci. (1994) [Pubmed]
  4. A common RNA recognition motif identified within a defined U1 RNA binding domain of the 70K U1 snRNP protein. Query, C.C., Bentley, R.C., Keene, J.D. Cell (1989) [Pubmed]
  5. Sequence of three copies of the gene for the major Drosophila heat shock induced protein and their flanking regions. Ingolia, T.D., Craig, E.A., McCarthy, B.J. Cell (1980) [Pubmed]
  6. Sequence organization of two recombinant plasmids containing genes for the major heat shock-induced protein of D. melanogaster. Craig, E.A., McCarthy, B.J., Wadsworth, S.C. Cell (1979) [Pubmed]
  7. Sex-lethal splicing autoregulation in vivo: interactions between SEX-LETHAL, the U1 snRNP and U2AF underlie male exon skipping. Nagengast, A.A., Stitzinger, S.M., Tseng, C.H., Mount, S.M., Salz, H.K. Development (2003) [Pubmed]
  8. The Drosophila sex determination gene snf encodes a nuclear protein with sequence and functional similarity to the mammalian U1A snRNP protein. Flickinger, T.W., Salz, H.K. Genes Dev. (1994) [Pubmed]
  9. Functioning of the Drosophila integral U1/U2 protein Snf independent of U1 and U2 small nuclear ribonucleoprotein particles is revealed by snf(+) gene dose effects. Cline, T.W., Rudner, D.Z., Barbash, D.A., Bell, M., Vutien, R. Proc. Natl. Acad. Sci. U.S.A. (1999) [Pubmed]
  10. Association of individual hnRNP proteins and snRNPs with nascent transcripts. Matunis, E.L., Matunis, M.J., Dreyfuss, G. J. Cell Biol. (1993) [Pubmed]
  11. A conserved epitope on a subset of SR proteins defines a larger family of Pre-mRNA splicing factors. Neugebauer, K.M., Stolk, J.A., Roth, M.B. J. Cell Biol. (1995) [Pubmed]
  12. Modulation of msl-2 5' splice site recognition by Sex-lethal. Förch, P., Merendino, L., Martínez, C., Valcárcel, J. RNA (2001) [Pubmed]
  13. Small nuclear ribonucleoprotein complexes of Drosophila melanogaster. Wooley, J.C., Cone, R.D., Tartof, D., Chung, S.Y. Proc. Natl. Acad. Sci. U.S.A. (1982) [Pubmed]
  14. An RNA splicing enhancer-like sequence is a component of a splicing inhibitor element from Rous sarcoma virus. McNally, L.M., McNally, M.T. Mol. Cell. Biol. (1998) [Pubmed]
  15. The Drosophila U1-70K protein is required for viability, but its arginine-rich domain is dispensable. Salz, H.K., Mancebo, R.S., Nagengast, A.A., Speck, O., Psotka, M., Mount, S.M. Genetics (2004) [Pubmed]
  16. Both loss-of-function and gain-of-function mutations in snf define a role for snRNP proteins in regulating Sex-lethal pre-mRNA splicing in Drosophila development. Salz, H.K., Flickinger, T.W. Genetics (1996) [Pubmed]
  17. Modulation of P-element pre-mRNA splicing by a direct interaction between PSI and U1 snRNP 70K protein. Labourier, E., Adams, M.D., Rio, D.C. Mol. Cell (2001) [Pubmed]
  18. Structure and expression of the Drosophila melanogaster gene for the U1 small nuclear ribonucleoprotein particle 70K protein. Mancebo, R., Lo, P.C., Mount, S.M. Mol. Cell. Biol. (1990) [Pubmed]
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