Modomics - A Database of RNA Modifications

Full name: rRNA 2′-O-methyltransferase fibrillarin
Synonym: FIB1, FLRN, fibrillarin
GI: 12056465
Orf:
COG: COG1889
UniProt: P22087
Structures: | 2IPX |
Complex: C/D RNP
Enzyme type: methyltransferase
Position of modification - modification: l:many - Xm
s:many - Xm

Comments:

FBL (fibrillarin) is a catalytic subunit of a multiprotein C/D RNP complex and homologue of S. cerevisiae Nop1 protein. It requires snoRNA to identify the substrate. The core of the complex is made of 15.5kD, Nop56 and Nop58 proteins.

Protein sequence:

MKPGFSPRGGGFGGRGGFGDRGGRGGRGGFGGGRGRGGGFRGRGRGGGGGGGGGGGGGRGGGGFHSGGNRGRGRGGKRGNQSGKNVMVEPHRHEGVFICR
GKEDALVTKNLVPGESVYGEKRVSISEGDDKIEYRAWNPFRSKLAAAILGGVDQIHIKPGAKVLYLGAASGTTVSHVSDIVGPDGLVYAVEFSHRSGRDL
INLAKKRTNIIPVIEDARHPHKYRMLIAMVDVIFADVAQPDQTRIVALNAHTFLRNGGHFVISIKANCIDSTASAEAVFASEVKKMQQENMKPQEQLTLE
PYERDHAVVVGVYRPPPKVKN


Diseases connected to this enzyme:

Description Reaction Disease Name
Hypermethylation of 4506 in 28S subunit of the ribosome leads to ribosome biogenesis deregulation xX:Xm
β-thalassemia
Loss of 2'-O-Me results in decreased leukemia self-renewal potential and reduced colony formation potential. xX:Xm
Leukemia
Hypermethylation of 4506 in 28S subunit of the ribosome leads to ribosome biogenesis deregulation xX:Xm
Hemoglobin E
Genetic associations and regulation of expression indicate an independent role for 14q32 snoRNAs in human cardiovascular disease. xX:Xm
Cardiovascular disease
Methylation levels on 5.8S, 18S, and 28S rRNAs are slightly increasing in p53 knock-downs. xX:Xm
Breast cancer

Publications:

Title Authors Journal Details PubMed Id DOI
Evolutionary conservation of the human nucleolar protein fibrillarin and its functional expression in yeast. Jansen RP, Hurt EC, Kern H, Lehtonen H, Carmo-Fonseca M, Lapeyre B, Tollervey D J Cell Biol [details] 2026646 -
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. Baserga SJ, Yang XD, Steitz JA EMBO J [details] 1714385 -
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. Yanagida M, Hayano T, Yamauchi Y, Shinkawa T, Natsume T, Isobe T, Takahashi N J Biol Chem [details] 14583623 -
cDNA cloning and sequencing of human fibrillarin, a conserved nucleolar protein recognized by autoimmune antisera. Aris JP, Blobel G Proc Natl Acad Sci U S A [details] 1846968 -

Links:

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