NP_001427.2
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NCBI GenBank Nucleotide #
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UniProt Primary Accession #
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UniProt Secondary Accession #
UniProt Related Accession #
NCBI Official Full Name
rRNA 2'-O-methyltransferase fibrillarin
NCBI Official Synonym Full Names
fibrillarin
NCBI Protein Information
rRNA 2'-O-methyltransferase fibrillarin
UniProt Protein Name
rRNA 2'-O-methyltransferase fibrillarin
UniProt Synonym Protein Names
34 kDa nucleolar scleroderma antigen; Histone-glutamine methyltransferase
UniProt Synonym Gene Names
NCBI Summary for FBL
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]
UniProt Comments for FBL
S-adenosyl-L-methionine-dependent methyltransferase that has the ability to methylate both RNAs and proteins. Involved in pre-rRNA processing by catalyzing the site-specific 2'-hydroxyl methylation of ribose moieties in pre-ribosomal 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. Also acts as a protein methyltransferase by mediating methylation of 'Gln-105' of histone H2A (H2AQ104me), a modification that impairs binding of the FACT complex and is specifically present at 35S ribosomal DNA locus (PubMed:24352239).
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Pathways associated with anti-FBL antibody
Diseases associated with anti-FBL antibody
Organs/Tissues associated with anti-FBL antibody
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