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Gene Review

FBL  -  fibrillarin

Homo sapiens

Synonyms: 34 kDa nucleolar scleroderma antigen, FIB, FIB1, FLRN, Histone-glutamine methyltransferase, ...
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Disease relevance of FBL

  • This protein is expressed at high levels by a Friend virus-induced erythroleukemia of C57BL/6 (B6) origin, FBL, and has been shown to serve as an efficient tumor-specific rejection antigen in B6 mice [1].
  • FBL -L reacted with leukemic cells in 8/9 cases of clinically recognized acute myeloid leukemia, including myeloid blast crisis of chronic granulocytic leukemia, 3/3 cases of chronic phase chronic myelogenous leukemia, and in 2/7 cases of clinically undifferentiated acute leukemia [2].
  • Four hundred forty-seven women attending a breast clinic because of either suspicious lesions, anxiety about breast cancer, follow-up after the removal of a benign breast lesion, or a family history of breast cancer had a routine test for percentage of ferritin-bearing lymphocytes ( FBL ) in their peripheral blood [3].
  • Autoantibodies specific against fibrillarin, a 34-kD nucleolar protein associated with U3-snRNP, are present in patients with systemic sclerosis (SSc) [4].
  • While a hexapeptide sequence NH2 terminus of fibrillarin is shared with an Epstein-Barr virus-encoded nuclear antigen, the COOH-terminal region shares sequence homology with P40, the capsid protein encoded by herpes virus type 1 [4].

Psychiatry related information on FBL

  • We show that a synthetic peptide R9 (GGRGRGGGF) with the RGG sequence present in human fibrillarin and fragile X mental retardation protein (FMRP) can be specifically methylated by rat brain extract [5].

High impact information on FBL

  • We present the primary sequence of Xenopus U8, a fibrillarin-associated nucleolar snRNA, and examine its expression through oocyte development [6].
  • The coiled body is a nuclear organelle that contains snRNPs involved in splicing, the non-snRNP splicing factor U2AF and the nucleolar protein fibrillarin [7].
  • To understand the mechanisms involved in the induction of these autoantibodies, we prepared a series of human fibrillarin recombinant proteins covering the entire molecule and analyzed their interaction with the autoantibodies present in various connective tissue diseases [4].
  • However, adoptively transferred envelope-specific T cells from immunized non-TG B6 mice mediated complete eradication of FBL tumor cells in TG mice, and did not induce detectable autoimmune damage to TG lymphoid tissues [1].
  • In vitro assembly of an archaeal box C/D sRNP using recombinant core proteins L7, Nop56/58 and fibrillarin has yielded an RNA:protein enzyme that guides methylation from both the terminal box C/D core and internal C'/D' RNP complexes [8].

Biological context of FBL


Anatomical context of FBL


Associations of FBL with chemical compounds


Physical interactions of FBL

  • Very little SRP RNA was detected in fibrillar centers or the DFC of the nucleolus, as defined by the RNA polymerase I-specific upstream binding factor and the protein fibrillarin, respectively [18].

Co-localisations of FBL


Other interactions of FBL

  • Coimmunoprecipitations demonstrate that the SMN complex associates with fibrillarin and with GAR1 in vivo [14].
  • Coimmunoprecipitation experiments confirmed that p68 and fibrillarin can form complexes in cellular extracts, and deletion analysis identified regions in each protein responsible for mediating the interaction [20].
  • Arginine methylation of recombinant murine fibrillarin by protein arginine methyltransferase [21].
  • Double immunostaining with p14(ARF) and B23/nucleophosmin or fibrillarin antibodies using 3D microscopy revealed that p14(ARF) is located mainly in the granular component of the nucleolus. p14(ARF) was also found in distinct granular aggregates scattered throughout the nucleoplasm [22].
  • To identify the structural elements responsible for these large conformational differences, we refined a crystal structure of Archaeoglobus fulgidus fibrillarin-Nop5p binary complex at 3.5 A [11].

Analytical, diagnostic and therapeutic context of FBL

  • Our results of protease digestion, methylation competition reactions, and immunoblotting with a methylarginine-specific antibody all indicate that the methylation of fibrillarin is in the N-terminal GAR domain and arginyl residues are modified [21].
  • In situ hybridization assays showed that the fibrillarin tagged by the elicited antibodies was colocalized with U3 snoRNP in the nucleolus in a clumpy manner and coprecipitated the U3 snoRNP [23].
  • The recombinant fibrillarin tested by ELISA was recognized by the clumpy scleroderma serum from the majority of patients [23].
  • Immunoprecipitation experiments using whole cell extracts of HeLa cells and cultured neurons revealed that abSMN coprecipitated small amounts of the U3 small nucleolar RNA (snoRNA) previously shown to be associated with fibrillarin in vivo [24].
  • Results obtained from competitive inhibition radioimmunoassay and Western blot analyses with purified recombinant fusion proteins revealed that these autoantibodies react primarily with epitope(s) present in the NH2- (AA 1-80) and COOH-terminal (AA 276-321) domains of fibrillarin [4].


  1. An evaluation of the potential to use tumor-associated antigens as targets for antitumor T cell therapy using transgenic mice expressing a retroviral tumor antigen in normal lymphoid tissues. Hu, J., Kindsvogel, W., Busby, S., Bailey, M.C., Shi, Y.Y., Greenberg, P.D. J. Exp. Med. (1993) [Pubmed]
  2. Analysis of myelomonocytic leukemic differentiation by a cell surface marker panel including a fucose-binding lectin from Lotus tetragonolobus. Elias, L., Van Epps, D.E. Blood (1984) [Pubmed]
  3. Ferritin-bearing lymphocytes in the diagnosis of breast cancer. Moroz, C., Kan, M., Chaimof, C., Marcus, H., Kupfer, B., Cuckle, H.S. Cancer (1984) [Pubmed]
  4. Antifibrillarin autoantibodies present in systemic sclerosis and other connective tissue diseases interact with similar epitopes. Kasturi, K.N., Hatakeyama, A., Spiera, H., Bona, C.A. J. Exp. Med. (1995) [Pubmed]
  5. Arginine methylation of a glycine and arginine rich peptide derived from sequences of human FMRP and fibrillarin. Ai, L.S., Lin, C.H., Hsieh, M., Li, C. Proc. Natl. Sci. Counc. Repub. China B (1999) [Pubmed]
  6. Disruption of U8 nucleolar snRNA inhibits 5.8S and 28S rRNA processing in the Xenopus oocyte. Peculis, B.A., Steitz, J.A. Cell (1993) [Pubmed]
  7. The coiled body. Lamond, A.I., Carmo-Fonseca, M. Trends Cell Biol. (1993) [Pubmed]
  8. Efficient RNA 2'-O-methylation requires juxtaposed and symmetrically assembled archaeal box C/D and C'/D' RNPs. Tran, E.J., Zhang, X., Maxwell, E.S. EMBO J. (2003) [Pubmed]
  9. Protein N-arginine methylation in subcellular fractions of lymphoblastoid cells. Lin, C.H., Hsieh, M., Li, Y.C., Li, S.Y., Pearson, D.L., Pollard, K.M., Li, C. J. Biochem. (2000) [Pubmed]
  10. Nucleolin and fibrillarin expression in stimulated lymphocytes and differentiating HL-60 cells. A flow cytometric assay. Méhes, G., Pajor, L. Cell Prolif. (1995) [Pubmed]
  11. Structural and thermodynamic evidence for a stabilizing role of Nop5p in S-adenosyl-L-methionine binding to fibrillarin. Aittaleb, M., Visone, T., Fenley, M.O., Li, H. J. Biol. Chem. (2004) [Pubmed]
  12. The coiled-coil domain of the Nop56/58 core protein is dispensable for sRNP assembly but is critical for archaeal box C/D sRNP-guided nucleotide methylation. Zhang, X., Champion, E.A., Tran, E.J., Brown, B.A., Baserga, S.J., Maxwell, E.S. RNA (2006) [Pubmed]
  13. Nucleocytoplasmic sorting of macromolecules following mitosis: fate of nuclear constituents after inhibition of pore complex function. Benavente, R., Scheer, U., Chaly, N. Eur. J. Cell Biol. (1989) [Pubmed]
  14. The survival of motor neurons (SMN) protein interacts with the snoRNP proteins fibrillarin and GAR1. Pellizzoni, L., Baccon, J., Charroux, B., Dreyfuss, G. Curr. Biol. (2001) [Pubmed]
  15. Nerve growth factor-mediated increases in protein methylation occur predominantly at type I arginine methylation sites and involve protein arginine methyltransferase 1. Cimato, T.R., Tang, J., Xu, Y., Guarnaccia, C., Herschman, H.R., Pongor, S., Aletta, J.M. J. Neurosci. Res. (2002) [Pubmed]
  16. A contribution to the incidence of nucleoli in normal human blood monocytes. Smetana, K., Jirásková, I., Turek, P., Chan, P.K., Busch, H. Haematologica (1997) [Pubmed]
  17. Double immunolocalization of major nucleolar proteins, fibrillarin and B23, in dividing mammalian cultured cells. Mukharyamova, K.S., Doudnik, O.A., Speransky, A.I., Zatsepina, O.V. Membrane & cell biology. (1999) [Pubmed]
  18. Signal recognition particle RNA localization within the nucleolus differs from the classical sites of ribosome synthesis. Politz, J.C., Lewandowski, L.B., Pederson, T. J. Cell Biol. (2002) [Pubmed]
  19. M phase phosphoprotein 10 is a human U3 small nucleolar ribonucleoprotein component. Westendorf, J.M., Konstantinov, K.N., Wormsley, S., Shu, M.D., Matsumoto-Taniura, N., Pirollet, F., Klier, F.G., Gerace, L., Baserga, S.J. Mol. Biol. Cell (1998) [Pubmed]
  20. The nuclear DEAD box RNA helicase p68 interacts with the nucleolar protein fibrillarin and colocalizes specifically in nascent nucleoli during telophase. Nicol, S.M., Causevic, M., Prescott, A.R., Fuller-Pace, F.V. Exp. Cell Res. (2000) [Pubmed]
  21. Arginine methylation of recombinant murine fibrillarin by protein arginine methyltransferase. Lin, C.H., Huang, H.M., Hsieh, M., Pollard, K.M., Li, C. J. Protein Chem. (2002) [Pubmed]
  22. Immunolocalization of human p14(ARF) to the granular component of the interphase nucleolus. Lindström, M.S., Klangby, U., Inoue, R., Pisa, P., Wiman, K.G., Asker, C.E. Exp. Cell Res. (2000) [Pubmed]
  23. U3 snoRNP associates with fibrillarin a component of the scleroderma clumpy nucleolar domain. Herrera-Esparza, R., Kruse, L., von Essen, M., Campos, L., Barbosa, O., Bollain, J.J., Badillo, I., Avalos-Díaz, E. Arch. Dermatol. Res. (2002) [Pubmed]
  24. Survival motor neuron protein in the nucleolus of mammalian neurons. Wehner, K.A., Ayala, L., Kim, Y., Young, P.J., Hosler, B.A., Lorson, C.L., Baserga, S.J., Francis, J.W. Brain Res. (2002) [Pubmed]
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