Gene Symbol: Fbl
Description: fibrillarin
Alias: AL022665, FIB, FLRN, RNU3IP1, rRNA 2'-O-methyltransferase fibrillarin, histone-glutamine methyltransferase, nucleolar protein 1
Species: mouse
Products:     Fbl

Top Publications

  1. Baran V, Vesela J, Rehak P, Koppel J, Flechon J. Localization of fibrillarin and nucleolin in nucleoli of mouse preimplantation embryos. Mol Reprod Dev. 1995;40:305-10 pubmed
    ..Our results confirm the view that the cortical region of NPBs could represent a nucleolonemal area involved in the resumption of nucleolar transcription in the early mouse embryo. ..
  2. Newton K, Petfalski E, Tollervey D, Caceres J. Fibrillarin is essential for early development and required for accumulation of an intron-encoded small nucleolar RNA in the mouse. Mol Cell Biol. 2003;23:8519-27 pubmed
    ..However, the level of the intron-encoded box C/D snoRNA U76 was clearly reduced. This suggests a high requirement for snoRNA synthesis during an early stage in development. ..
  3. Zhang J, Tomasini A, Mayer A. RBM19 is essential for preimplantation development in the mouse. BMC Dev Biol. 2008;8:115 pubmed publisher
    ..The data indicate that RBM19 is essential for preimplantation development, highlighting the importance of de novo nucleologenesis during this critical developmental stage. ..
  4. Romanova L, Anger M, Zatsepina O, Schultz R. Implication of nucleolar protein SURF6 in ribosome biogenesis and preimplantation mouse development. Biol Reprod. 2006;75:690-6 pubmed
    ..These results suggest that Surf6 is essential for mouse preimplantation development, presumably by regulating ribosome biogenesis...
  5. Cuadros Fernández J, Esponda P. Immunocytochemical localisation of the nucleolar protein fibrillarin and RNA polymerase I during mouse early embryogenesis. Zygote. 1996;4:49-58 pubmed
    ..8% of injected embryos reach the blastocyst stage. ..
  6. Wu X, Sandhu S, Patel N, Triggs Raine B, Ding H. EMG1 is essential for mouse pre-implantation embryo development. BMC Dev Biol. 2010;10:99 pubmed publisher
    ..Further, our findings also lend support to the previous study that showed Bowen-Conradi syndrome results from a partial EMG1 deficiency. A complete deficiency would not be expected to be compatible with a live birth. ..
  7. Horáková A, Bartova E, Galiova G, Uhlírová R, Matula P, Kozubek S. SUV39h-independent association of HP1 beta with fibrillarin-positive nucleolar regions. Chromosoma. 2010;119:227-41 pubmed publisher
    ..Moreover, the presence of HP1 beta in nucleoli is likely connected with transcription of ribosomal genes and with the role of fibrillarin in nucleolar processes...
  8. Ferreira J, Carmo Fonseca M. The biogenesis of the coiled body during early mouse development. Development. 1995;121:601-12 pubmed
    ..Our results suggest that the embryonic 'nucleolus-like body' is a structural scaffold that nucleates independently the formation of the coiled body and the assembly of the machinery responsible for ribosome biosynthesis. ..
  9. Hilditch Maguire P, Trettel F, Passani L, Auerbach A, Persichetti F, MacDonald M. Huntingtin: an iron-regulated protein essential for normal nuclear and perinuclear organelles. Hum Mol Genet. 2000;9:2789-97 pubmed
    ..Thus, organelles that require huntingtin to function suggest roles for the protein in RNA biogenesis, trafficking and iron homeostasis to be explored in HD pathogenesis. ..

More Information


  1. Bonetti P, Davoli T, Sironi C, Amati B, Pelicci P, Colombo E. Nucleophosmin and its AML-associated mutant regulate c-Myc turnover through Fbw7 gamma. J Cell Biol. 2008;182:19-26 pubmed publisher
    ..Because Fbw7 induces degradation of other growth-promoting proteins, the NPM-Fbw7 interaction emerges as a central tumor suppressor mechanism in human cancer. ..
  2. Brady S, Yu Y, Maggi L, Weber J. ARF impedes NPM/B23 shuttling in an Mdm2-sensitive tumor suppressor pathway. Mol Cell Biol. 2004;24:9327-38 pubmed
    ..By actively sequestering NPM in the nucleolus, ARF utilizes an additional mechanism of tumor suppression, one that is readily antagonized by Mdm2. ..
  3. Yang Z, Jakymiw A, Wood M, Eystathioy T, Rubin R, Fritzler M, et al. GW182 is critical for the stability of GW bodies expressed during the cell cycle and cell proliferation. J Cell Sci. 2004;117:5567-78 pubmed
  4. Shishova K, Khodarovich Y, Lavrentyeva E, Zatsepina O. [Analysis of the Localization of Fibrillarin and Sites of Pre-rRNA Synthesis in the Nucleolus-Like Bodies of Mouse GV Oocytes after Mild Treatment with Proteinase K]. Ontogenez. 2015;46:162-73 pubmed
    ..The fluorescent dye FITC can be recommended for assessment of intracellular protein localization in the oocytes of all mammalian species. ..
  5. Burns K, Viveiros M, Ren Y, Wang P, DeMayo F, Frail D, et al. Roles of NPM2 in chromatin and nucleolar organization in oocytes and embryos. Science. 2003;300:633-6 pubmed
    ..Thus, Npm2 is a maternal effect gene critical for nuclear and nucleolar organization and embryonic development. ..
  6. Guetg C, Lienemann P, Sirri V, Grummt I, Hernandez Verdun D, Hottiger M, et al. The NoRC complex mediates the heterochromatin formation and stability of silent rRNA genes and centromeric repeats. EMBO J. 2010;29:2135-46 pubmed publisher
    ..These findings point to a role of TIP5 in protecting genome stability and suggest that it can play a role in the cellular transformation process. ..
  7. Mekhail K, Khacho M, Carrigan A, Hache R, Gunaratnam L, Lee S. Regulation of ubiquitin ligase dynamics by the nucleolus. J Cell Biol. 2005;170:733-44 pubmed
    ..Data shown here provide the first evidence that cells have evolved a mechanism to regulate molecular networks by reversibly switching proteins between a mobile and static state. ..
  8. Sugihara Y, Sadohara E, Yonezawa K, Kugo M, Oshima K, Matsuda T, et al. Identification and expression of an autosomal paralogue of ribosomal protein S4, X-linked, in mice: potential involvement of testis-specific ribosomal proteins in translation and spermatogenesis. Gene. 2013;521:91-9 pubmed publisher
  9. Sandilands A, Hutcheson A, Long H, Prescott A, Vrensen G, Löster J, et al. Altered aggregation properties of mutant gamma-crystallins cause inherited cataract. EMBO J. 2002;21:6005-14 pubmed
    ..The mutant gamma-crystallins initially disrupt nuclear function, but then this progresses to a full cataract phenotype. ..
  10. Chen M, Rockel T, Steinweger G, Hemmerich P, Risch J, von Mikecz A. Subcellular recruitment of fibrillarin to nucleoplasmic proteasomes: implications for processing of a nucleolar autoantigen. Mol Biol Cell. 2002;13:3576-87 pubmed
    ..The latter observation might constitute the cell biological basis of autoimmune responses that specifically target fibrillarin in mercury-mouse models and scleroderma. ..
  11. Fischer H, Zhang X, O Brien K, Kylsten P, Engvall E. C7, a novel nucleolar protein, is the mouse homologue of the Drosophila late puff product L82 and an isoform of human OXR1. Biochem Biophys Res Commun. 2001;281:795-803 pubmed
    ..Antibodies to recombinant C7 protein localized to nucleoli in a variety of cell types, suggesting that C7 may be involved in the formation or function of this important organelle. ..
  12. Turley S, Tan E, Pollard K. Molecular cloning and sequence analysis of U3 snoRNA-associated mouse fibrillarin. Biochim Biophys Acta. 1993;1216:119-22 pubmed
    ..Specific amino acid residues in yeast NOP1 thought to be associated with the synthesis and maturation of ribosomes show strong conservation between the mouse, human, amphibian and yeast protein sequences. ..
  13. Okano Y, Steen V, Medsger T. Autoantibody to U3 nucleolar ribonucleoprotein (fibrillarin) in patients with systemic sclerosis. Arthritis Rheum. 1992;35:95-100 pubmed
    ..These antibodies may identify one or more unusual clinical subsets of SSc. ..
  14. Hamdane N, Tremblay M, Dillinger S, Stefanovsky V, Nemeth A, Moss T. Disruption of the UBF gene induces aberrant somatic nucleolar bodies and disrupts embryo nucleolar precursor bodies. Gene. 2017;612:5-11 pubmed publisher
  15. Malatesta M, Caporaloni C, Gavaudan S, Rocchi M, Serafini S, Tiberi C, et al. Ultrastructural morphometrical and immunocytochemical analyses of hepatocyte nuclei from mice fed on genetically modified soybean. Cell Struct Funct. 2002;27:173-80 pubmed
    ..In conclusion, our data suggest that GM soybean intake can influence hepatocyte nuclear features in young and adult mice; however, the mechanisms responsible for such alterations remain unknown. ..
  16. Swartling F, Grimmer M, Hackett C, Northcott P, Fan Q, Goldenberg D, et al. Pleiotropic role for MYCN in medulloblastoma. Genes Dev. 2010;24:1059-72 pubmed publisher
    ..Targeted expression of MYCN thus contributes to initiation, progression, and maintenance of MB, suggesting a central role for MYCN in pathogenesis. ..
  17. Meng L, Yasumoto H, Tsai R. Multiple controls regulate nucleostemin partitioning between nucleolus and nucleoplasm. J Cell Sci. 2006;119:5124-36 pubmed
    ..Our work reveals that the partitioning of nucleostemin employs complex mechanisms involving both nucleolar and nucleoplasmic components, and provides insight into the post-translational regulation of its activity. ..
  18. Mas C, Guimiot Maloum I, Guimiot F, Khelfaoui M, Nepote V, Bourgeois F, et al. Molecular cloning and expression pattern of the Fkbp25 gene during cerebral cortical neurogenesis. Gene Expr Patterns. 2005;5:577-85 pubmed
    ..af135595.). ..
  19. Aris J, Blobel G. cDNA cloning and sequencing of human fibrillarin, a conserved nucleolar protein recognized by autoimmune antisera. Proc Natl Acad Sci U S A. 1991;88:931-5 pubmed
    ..This identity suggests the evolutionary conservation of an important function early in the pathway for ribosome biosynthesis. ..