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Gene Review

Rbl2  -  retinoblastoma-like 2

Rattus norvegicus

Synonyms: 130 kDa retinoblastoma-associated protein, PPAR-alpha-interacting complex protein 128, PRIC128, RBR-2, Retinoblastoma-like protein 2, ...
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Disease relevance of Rbl2

  • Fujinami sarcoma virus (FSV) encodes a transforming protein of 130,000 daltons (P130) which is associated with a tyrosine-specific protein kinase activity [1].
  • Microsome- and polysome-localized P130 and P123 were found in rat liver and Ac2F hepatoma cells [2].

High impact information on Rbl2

  • When injected at P30 or P45, virtually the same number of photoreceptors survived at P130 as when injected at P15 [3].
  • Both RB and RB2/P130 decreased cell proliferation rate [4].
  • Furthermore, a combination of ionic and nonionic detergents that effectively solubilized subcellular membranes was insufficient to solubilize P130 unless the salt concentration was raised [1].
  • At 10 mM NaCl, 70% of P130 sedimented in the large granule fraction, whereas at 500 mM NaCl 70 to 90% of P130 was recovered in the cytosol fraction [1].
  • Immunofluorescent staining of several FSV-transformed rat cell lines with a tumor regressor antiserum specific against the fps sequences of P130 showed that the major staining was localized in the cytoplasm [1].

Biological context of Rbl2

  • On the contrary, RB2/P130 increased the percentage of cells in apoptosis [5].
  • In MSCs that were either uncommitted or committed toward neural differentiation, we ectopically expressed RB and RB2/P130 genes and analyzed their role in regulating the cell cycle, apoptosis and differentiation [5].
  • On the other hand, in MSCs committed toward the neural phenotype, both RB and RB2/P130 reduced cell proliferation rate and affected the apoptotic process [5].
  • P130 comprising 845 amino acid residues possesses several functional domains and yields an electrophoretically distinctive isoform, P123, by altering its phosphorylation status in association with translocation across the nuclear membrane and from the digitonin-extractable fraction of the nucleus to the nuclear scaffold [2].

Anatomical context of Rbl2

  • Role of RB and RB2/P130 genes in marrow stromal stem cells plasticity [5].
  • Our previous work [Hibino et al. (1992) Biochem. Biophys. Res. Commun. 184, 853-858] has shown that a highly repetitive component in rat nuclear DNA forms a sequence-directed bend to have the binding affinity for the nuclear scaffold protein, P130 [6].

Associations of Rbl2 with chemical compounds

  • Distamycin A, an antibiotic which binds specifically to AT-rich DNA, removed the bend in the XmnI fragment and inhibited binding of the fragment to P123 or P130, whereas neither removal of the bend nor binding inhibition was observed with chromomycin A3, an antibiotic specific for GC-rich sites in DNA [7].
  • The immunoblot analysis with anti-phosphoamino acid antibodies suggested that phosphorylation of serine and threonine residues occurs on P123 and P130, but also of tyrosine residue(s) on P130 [8].

Other interactions of Rbl2

  • For the above reasons, RB and RB2/P130 may have a role in neural differentiation but not in neural determination [5].

Analytical, diagnostic and therapeutic context of Rbl2


  1. Cytoplasmic localization of the transforming protein of Fujinami sarcoma virus: salt-sensitive association with subcellular components. Feldman, R.A., Wang, E., Hanafusa, H. J. Virol. (1983) [Pubmed]
  2. Molecular properties and intracellular localization of rat liver nuclear scaffold protein P130. Hibino, Y., Usui, T., Morita, Y., Hirose, N., Okazaki, M., Sugano, N., Hiraga, K. Biochim. Biophys. Acta (2006) [Pubmed]
  3. Ribozyme rescue of photoreceptor cells in P23H transgenic rats: long-term survival and late-stage therapy. LaVail, M.M., Yasumura, D., Matthes, M.T., Drenser, K.A., Flannery, J.G., Lewin, A.S., Hauswirth, W.W. Proc. Natl. Acad. Sci. U.S.A. (2000) [Pubmed]
  4. RB and RB2/p130 genes demonstrate both specific and overlapping functions during the early steps of in vitro neural differentiation of marrow stromal stem cells. Jori, F.P., Melone, M.A., Napolitano, M.A., Cipollaro, M., Cascino, A., Giordano, A., Galderisi, U. Cell Death Differ. (2005) [Pubmed]
  5. Role of RB and RB2/P130 genes in marrow stromal stem cells plasticity. Jori, F.P., Napolitano, M.A., Melone, M.A., Cipollaro, M., Cascino, A., Giordano, A., Galderisi, U. J. Cell. Physiol. (2004) [Pubmed]
  6. Properties of a DNA-binding protein from rat nuclear scaffold fraction. Hibino, Y., Tsukada, S., Sugano, N. Biochem. Biophys. Res. Commun. (1993) [Pubmed]
  7. Purification and characterization of nuclear scaffold proteins which bind to a highly repetitive bent DNA from rat liver. Hibino, Y., Nakamura, K., Tsukada, S., Sugano, N. Biochim. Biophys. Acta (1993) [Pubmed]
  8. Involvement of phosphorylation in binding of nuclear scaffold proteins from rat liver to a highly repetitive DNA component. Hibino, Y., Ohzeki, H., Hirose, N., Sugano, N. Biochim. Biophys. Acta (1998) [Pubmed]
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