Fibrillarin

Fibrillarin, also known as FBL, is a human gene.cite web | title = Entrez Gene: FBL fibrillarin| url = http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=2091| accessdate = ]

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summary_text = This gene product is a component of a nucleolar small nuclear ribonucleoprotein (snRNP) particle thought to participate in the first step in processing preribosomal RNA. It is associated with the U3, U8, and U13 small nuclear RNAs and is located in the dense fibrillar component (DFC) of the nucleolus. The encoded protein contains an N-terminal repetitive domain that is rich in glycine and arginine residues, like fibrillarins in other species. Its central region resembles an RNA-binding domain and contains an RNP consensus sequence. Antisera from approximately 8% of humans with the autoimmune disease scleroderma recognize fibrillarin.cite web | title = Entrez Gene: FBL fibrillarin| url = http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=2091| accessdate = ]

Fibrillarin is a component of several ribonucleoproteins including a nucleolar small nuclear ribonucleoprotein (SnRNP) and one of the two classes of small nucleolar ribonucleoproteins (snoRNPs). SnRNAs function in RNA splicing while snoRNPs function in ribosomal RNA processing.

Fibrillarin is associated with U3, U8 and U13 small nuclear RNAs in mammals and is similar to the yeast NOP1 protein. Fibrillarin has a well conserved sequence of around 320 amino acids, and contains 3 domains, an N-terminal Gly/Arg-rich region; a central domain resembling other RNA-binding proteins and containing an RNP-2-like consensus sequence; and a C-terminal alpha-helical domain. An evolutionarily related pre-rRNA processing protein, which lacks the Gly/Arg-rich domain, has been found in various archaebacteria.

A study by Schultz et al indicated that the K-turn binding 15.5-kDa protein (called Snu13 in yeast) interacts with spliceosome proteins hPRP31, hPRP3, hPRP4, CYPH and the small nucleolar ribonucleoproteins NOP56, NOP58, and fibrillarin. The 15.5-kDa protein has sequence similarity to other RNA-binding proteins such as ribosomal proteins S12, L7a, and L30 and the snoRNP protein NHP2. The U4/U6 snRNP contains 15.5-kDa protein [ [http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pubmed&pubmedid=16782898 Protein-Protein and Protein-RNA Contacts both Contribute to the 15.5K-Mediated Assembly of the U4/U6 snRNP and the Box C/D snoRNPs] by Annemarie Schultz, Stephanie Nottrott, Nicholas James Watkins and Reinhard Lührmann in "Molecular and Cellular Biology" (2006) Volume 26, pages 5146–5154.] . The 15.5-kDa protein also exists in a ribonucleoprotein complex that binds the U3 box B/C motif. The 15.5-kDa protein also exists as one of the four core proteins of the C/D small nucleolar ribonucleoprotein that mediates methylation of pre-ribosomal RNAs.

Structural evidence supporting the idea that fibrillarin is the small nucleolar ribonucleoprotein methyltransferase has been reviewed [ [http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pubmed&pubmedid=17284456 The structure and function of small nucleolar ribonucleoproteins] by Steve L. Reichow, Tomoko Hamma, Adrian R. Ferré-D'Amaré and Gabriele Varani in "Nucleic Acids Research" (2007) Volume 35, pages 1452–1464.] .

References

Further reading

PBB_Further_reading
citations =
*cite journal | author=Baserga SJ, Yang XD, Steitz JA |title=An intact Box C sequence in the U3 snRNA is required for binding of fibrillarin, the protein common to the major family of nucleolar snRNPs. |journal=EMBO J. |volume=10 |issue= 9 |pages= 2645–51 |year= 1991 |pmid= 1714385 |doi=
*cite journal | author=Okano Y, Steen VD, Medsger TA |title=Autoantibody to U3 nucleolar ribonucleoprotein (fibrillarin) in patients with systemic sclerosis. |journal=Arthritis Rheum. |volume=35 |issue= 1 |pages= 95–100 |year= 1992 |pmid= 1731817 |doi=
*cite journal | author=Aris JP, Blobel G |title=cDNA cloning and sequencing of human fibrillarin, a conserved nucleolar protein recognized by autoimmune antisera. |journal=Proc. Natl. Acad. Sci. U.S.A. |volume=88 |issue= 3 |pages= 931–5 |year= 1991 |pmid= 1846968 |doi=
*cite journal | author=Jansen RP, Hurt EC, Kern H, "et al." |title=Evolutionary conservation of the human nucleolar protein fibrillarin and its functional expression in yeast. |journal=J. Cell Biol. |volume=113 |issue= 4 |pages= 715–29 |year= 1991 |pmid= 2026646 |doi=
*cite journal | author=Lischwe MA, Ochs RL, Reddy R, "et al." |title=Purification and partial characterization of a nucleolar scleroderma antigen (Mr = 34,000; pI, 8.5) rich in NG,NG-dimethylarginine. |journal=J. Biol. Chem. |volume=260 |issue= 26 |pages= 14304–10 |year= 1985 |pmid= 2414294 |doi=
*cite journal | author=Méhes G, Pajor L |title=Nucleolin and fibrillarin expression in stimulated lymphocytes and differentiating HL-60 cells. A flow cytometric assay. |journal=Cell Prolif. |volume=28 |issue= 6 |pages= 329–36 |year= 1995 |pmid= 7626687 |doi=
*cite journal | author=Liu Q, Dreyfuss G |title=A novel nuclear structure containing the survival of motor neurons protein. |journal=EMBO J. |volume=15 |issue= 14 |pages= 3555–65 |year= 1996 |pmid= 8670859 |doi=
*cite journal | author=Magoulas C, Zatsepina OV, Jordan PW, "et al." |title=The SURF-6 protein is a component of the nucleolar matrix and has a high binding capacity for nucleic acids in vitro. |journal=Eur. J. Cell Biol. |volume=75 |issue= 2 |pages= 174–83 |year= 1998 |pmid= 9548374 |doi=
*cite journal | author=Ai LS, Lin CH, Hsieh M, Li C |title=Arginine methylation of a glycine and arginine rich peptide derived from sequences of human FMRP and fibrillarin. |journal=Proc. Natl. Sci. Counc. Repub. China B |volume=23 |issue= 4 |pages= 175–80 |year= 1999 |pmid= 10518318 |doi=
*cite journal | author=Pintard L, Kressler D, Lapeyre B |title=Spb1p is a yeast nucleolar protein associated with Nop1p and Nop58p that is able to bind S-adenosyl-L-methionine in vitro. |journal=Mol. Cell. Biol. |volume=20 |issue= 4 |pages= 1370–81 |year= 2000 |pmid= 10648622 |doi=
*cite journal | author=Nicol SM, Causevic M, Prescott AR, Fuller-Pace FV |title=The nuclear DEAD box RNA helicase p68 interacts with the nucleolar protein fibrillarin and colocalizes specifically in nascent nucleoli during telophase. |journal=Exp. Cell Res. |volume=257 |issue= 2 |pages= 272–80 |year= 2000 |pmid= 10837141 |doi= 10.1006/excr.2000.4886
*cite journal | author=Pellizzoni L, Baccon J, Charroux B, Dreyfuss G |title=The survival of motor neurons (SMN) protein interacts with the snoRNP proteins fibrillarin and GAR1. |journal=Curr. Biol. |volume=11 |issue= 14 |pages= 1079–88 |year= 2001 |pmid= 11509230 |doi=
*cite journal | author=Zhou X, Tan FK, Xiong M, "et al." |title=Systemic sclerosis (scleroderma): specific autoantigen genes are selectively overexpressed in scleroderma fibroblasts. |journal=J. Immunol. |volume=167 |issue= 12 |pages= 7126–33 |year= 2001 |pmid= 11739535 |doi=
*cite journal | author=Andersen JS, Lyon CE, Fox AH, "et al." |title=Directed proteomic analysis of the human nucleolus. |journal=Curr. Biol. |volume=12 |issue= 1 |pages= 1–11 |year= 2002 |pmid= 11790298 |doi=
*cite journal | author=Cimato TR, Tang J, Xu Y, "et al." |title=Nerve growth factor-mediated increases in protein methylation occur predominantly at type I arginine methylation sites and involve protein arginine methyltransferase 1. |journal=J. Neurosci. Res. |volume=67 |issue= 4 |pages= 435–42 |year= 2002 |pmid= 11835310 |doi=
*cite journal | author=Fujiyama S, Yanagida M, Hayano T, "et al." |title=Isolation and proteomic characterization of human Parvulin-associating preribosomal ribonucleoprotein complexes. |journal=J. Biol. Chem. |volume=277 |issue= 26 |pages= 23773–80 |year= 2002 |pmid= 11960984 |doi= 10.1074/jbc.M201181200
*cite journal | author=Whitehead SE, Jones KW, Zhang X, "et al." |title=Determinants of the interaction of the spinal muscular atrophy disease protein SMN with the dimethylarginine-modified box H/ACA small nucleolar ribonucleoprotein GAR1. |journal=J. Biol. Chem. |volume=277 |issue= 50 |pages= 48087–93 |year= 2003 |pmid= 12244096 |doi= 10.1074/jbc.M204551200
*cite journal | author=Herrera-Esparza R, Kruse L, von Essen M, "et al." |title=U3 snoRNP associates with fibrillarin a component of the scleroderma clumpy nucleolar domain. |journal=Arch. Dermatol. Res. |volume=294 |issue= 7 |pages= 310–7 |year= 2003 |pmid= 12373336 |doi= 10.1007/s00403-002-0338-7
*cite journal | author=Chen M, Rockel T, Steinweger G, "et al." |title=Subcellular recruitment of fibrillarin to nucleoplasmic proteasomes: implications for processing of a nucleolar autoantigen. |journal=Mol. Biol. Cell |volume=13 |issue= 10 |pages= 3576–87 |year= 2003 |pmid= 12388758 |doi= 10.1091/mbc.02-05-0083
*cite journal | author=Watkins NJ, Dickmanns A, Lührmann R |title=Conserved stem II of the box C/D motif is essential for nucleolar localization and is required, along with the 15.5K protein, for the hierarchical assembly of the box C/D snoRNP. |journal=Mol. Cell. Biol. |volume=22 |issue= 23 |pages= 8342–52 |year= 2003 |pmid= 12417735 |doi=

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