Protein Name fibrillarin
Organism Homo sapiens
Gene ID 2091
Gene Symbol


UniProt P22087 (FBRL_HUMAN)
Relationships Total Number of functionally related compound(s) : 33
Total Number of Articles : 30


Gene Summary

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. [provided by RefSeq, Jul 2008]

  • rRNA 2'-O-methyltransferase fibrillarin
  • 34 kDa nucleolar scleroderma antigen
  • 34-kD nucleolar scleroderma antigen
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