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Fibrillarin, encoded by the FBL gene (also known as FBLL1), is a highly conserved nucleolar protein that plays a critical role in the biogenesis of ribosomes. It is a component of the small nucleolar ribonucleoprotein (snoRNP) complexes and is primarily involved in the chemical modification of ribosomal RNA (rRNA), specifically in the methylation of the 2'-O-ribose residues of rRNA. This modification is essential for the proper processing and maturation of pre-rRNA into functional ribosomal subunits, which are crucial for protein synthesis within the cell. Fibrillarin is not only important for ribosome production but also for maintaining the structural integrity of the nucleolus, the site of ribosome assembly. Dysregulation or mutations in the FBL gene have been associated with various human diseases, including cancer, where altered ribosome biogenesis can contribute to uncontrolled cell proliferation. Additionally, fibrillarin has been studied in the context of autoimmune diseases, such as systemic sclerosis, where it can serve as an autoantigen. Understanding the function and regulation of fibrillarin is vital for insights into cellular growth, protein synthesis, and the pathogenesis of related diseases.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
Protein Aliases: 34 kDa nucleolar scleroderma antigen; 34-kD nucleolar scleroderma antigen; Histone-glutamine methyltransferase; Nucleolar protein 1; Protein-glutamine methyltransferase; RNA, U3 small nucleolar interacting protein 1; rRNA 2'-O-methyltransferase fibrillarin; rRNA/tRNA 2'-O-methyltransferase fibrillarin-like protein 1; U6 snRNA 2'-O-methyltransferase fibrillarin
Gene Aliases: AI595406; AL022665; FBL; Fbll1; FIB; FIB1; FLRN; RGD1562841; RNU3IP1
UniProt ID: (Human) P22087, (Rat) P22509, (Mouse) P35550, (Mouse) Q80WS3
Entrez Gene ID: (Human) 2091, (Rat) 292747, (Mouse) 14113, (Mouse) 237730, (Rat) 363563
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