Clone: A00153.01

IgG Subclass: Mouse Monoclonal Specificity: Cross reacts with fibrillarin in human. rat. plant. Drosophila. C. elegans and S. pombe. Application: Dilution for immunoblot and ELISA range: 1:2.500 to 1:20.000. Nop1p/Fibrillarin was originally identified as a nucleolar protein of bakers yeast. Saccharomyces cerevisiae (accession P15646). The Nop1p protein is essential for yeast viability and is localized in the nucleoli. The human homologue of Nop1p is fibrillar

Function: Involved in pre-rRNA processing. Utilizes the methyl donor S-adenosyl-L-methionine to catalyze the site-specific 2\'-hydroxyl methylation of ribose moieties in preribosomal RNA. Site specificity is provided by a guide RNA that base pairs with the substrate. Methylation occurs at a characteristic distance from the sequence involved in base pairing with the guide RNA.

Subunit: Interacts with NOLC1 (By similarity). Component of box C/D small nucleolar ribonucleoprotein (snoRNP) particles that contain NHP2L1, FBL, NOP5 and NOL5A, plus a guide RNA. It is associated with the U3, U8, U13, X and Y small nuclear RNAs. Component of several ribosomal and nucleolar protein complexes. Interacts with PRMT5.

Subcellular Location: Nucleus; nucleolus. Fibrillar region of the nucleolus.

Ptm: By homology to other fibrillarins, some or all of the N-terminal domain arginines are modified to asymmetric dimethylarginine (DMA).

Disease: Antisera from circa 8% of humans with the autoimmune disease scleroderma recognize fibrillarin.

Similarity: Belongs to the fibrillarin family. [1] Jansen,R.P., Hurt,E.C., Kern,H., Lehtonen,H., Carmo-Fonseca,M., Lapeyre,B. and Tollervey,D.
Evolutionary conservation of the human nucleolar protein fibrillarin and its functional expression in yeast
[2] Hurt,E.C., et al.
Direct Submission
[3] Aris J.P., Blobel G.
cDNA cloning and sequencing of human fibrillarin, a conserved nucleolar protein recognized by autoimmune antisera.
[4] Jansen R.P., Hurt E.C., Kern H., Lehtonen H., Carmo-Fonseca M., Lapeyre B., Tollervey D.
Evolutionary conservation of the human nucleolar protein fibrillarin and its functional expression in yeast.
[5] Kalnine N., Chen X., Rolfs A., Halleck A., Hines L., Eisenstein S., Koundinya M., Raphael J., Moreira D., Kelley T., et al.
Cloning of human full-length CDSs in BD Creator(TM) system donor vector.
[6] Grimwood J., Gordon L.A., Olsen A.S., Terry A., Schmutz J., Lamerdin J.E., Hellsten U., Goodstein D., Couronne O., Tran-Gyamfi M., et al.
The DNA sequence and biology of human chromosome 19.
[7] Lischwe M.A., Ochs R.L., Reddy R., Cook R.G., Yeoman L.C., Tan E.M., Reichlin M., Busch H.
Purification and partial characterization of a nucleolar scleroderma antigen (Mr = 34,000; pI, 8.5) rich in NG,NG-dimethylarginine.
[8] Baserga S.J., Yang X.D., Steitz J.A.
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.
[9] Yanagida M., Hayano T., Yamauchi Y., Shinkawa T., Natsume T., Isobe T., Takahashi N.
Human fibrillarin forms a sub-complex with splicing factor 2-associated p32, protein arginine methyltransferases, and tubulins alpha 3 and beta 1 that is independent of its association with preribosomal ribonucleoprotein complexes.