FBL
rRNA 2'-O-methyltransferase fibrillarin
Also known as: FBRL_HUMAN, FIB, FLRN, Nop1, RNU3IP1
Cross-references: UniProt · Ensembl · Human Protein Atlas · GeneCards · NCBI Gene
Protein identityUniProt · HPA
- UniProt accession
- P22087
- Gene
- FBL
- Ensembl
- ENSG00000105202
- Chromosome
- 19
- Canonical length
- 321 aa
- Protein class
- Plasma proteins, Predicted intracellular proteins
- Subcellular location
- Nucleoplasm,Nucleoli fibrillar center
OverviewNCBI Gene
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]
Canonical amino-acid sequenceUniProt
321 residues, UniProt reviewed canonical sequence.
>P22087|FBL
1 MKPGFSPRGG GFGGRGGFGD RGGRGGRGGF GGGRGRGGGF RGRGRGGGGG GGGGGGGGRG
61 GGGFHSGGNR GRGRGGKRGN QSGKNVMVEP HRHEGVFICR GKEDALVTKN LVPGESVYGE
121 KRVSISEGDD KIEYRAWNPF RSKLAAAILG GVDQIHIKPG AKVLYLGAAS GTTVSHVSDI
181 VGPDGLVYAV EFSHRSGRDL INLAKKRTNI IPVIEDARHP HKYRMLIAMV DVIFADVAQP
241 DQTRIVALNA HTFLRNGGHF VISIKANCID STASAEAVFA SEVKKMQQEN MKPQEQLTLE
301 PYERDHAVVV GVYRPPPKVK NLocalizationUniProt · AlphaFold · HPA
Whether an antibody against FBL can act on the native protein depends on physical access: surface and secreted proteins are reachable by circulating antibodies, intracellular proteins usually are not.
- Antibody reachability
- Intracellular
- Secreted
- No
- Transmembrane segments
- 0
- Mean surface accessibility (rSASA)
- 0.39
- Highest tissue expression
- 247 nTPM
Expression across tissuesHPA
Tissue
- ovary: 247 nTPM
- skin: 214 nTPM
- esophagus: 186 nTPM
- cervix: 165 nTPM
- vagina: 154 nTPM
- pancreas: 150 nTPM
Single-cell type
- late spermatids: 855 nCPM
- early spermatids: 402 nCPM
- esophageal basal cells: 377 nCPM
- extravillous trophoblasts: 281 nCPM
- enteric stem cells: 272 nCPM
- basal keratinocytes: 261 nCPM
Immune cell
- MAIT T-cell: 102 nTPM
- naive CD4 T-cell: 101 nTPM
- naive CD8 T-cell: 98 nTPM
- NK-cell: 93 nTPM
- naive B-cell: 85 nTPM
- memory B-cell: 84 nTPM
Brain region
- thalamus: 23 nTPM
- white matter: 23 nTPM
- basal ganglia: 22 nTPM
- medulla oblongata: 22 nTPM
- spinal cord: 21 nTPM
- amygdala: 20 nTPM
DiseaseUniProt · ClinVar · IEDB · PubMed
Four sources answering four different questions about FBL.
Disease | ImmuneIEDB
Conditions an epitope on FBL was assayed in.
- systemic scleroderma B cell
Disease | AutoantibodyPubMed
Conditions in which antibodies against FBL are reported. Each links to that disease's full target list.
ReferencesPubMed · IEDB
Publications for FBL from three distinct lines of evidence, kept separate because they answer different questions: whether antibodies are directed at the protein, whether a B-cell epitope has been mapped on it, and whether a T-cell epitope has. Each is labelled with its source.
Reference: AutoantibodyPubMed
68 publications
- The clinical relevance of autoantibodies in scleroderma.
2003 · Arthritis Res Ther · RCR 6.1 · 243 citations - Murine susceptibility to mercury. I. Autoantibody profiles and systemic immune deposits in inbred, congenic, and intra-H-2 recombinant strains.
1992 · Clin Immunol Immunopathol · RCR 3.9 · 132 citations - Autoantibodies to fibrillarin in systemic sclerosis (scleroderma). An immunogenetic, serologic, and clinical analysis.
1996 · Arthritis Rheum · RCR 3.7 · 144 citations - Anti-fibrillarin autoantibodies in mercury-treated mice.
1989 · Clin Exp Immunol · RCR 3.1 · 104 citations - 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.
1991 · EMBO J · RCR 2.9 · 152 citations
Show 20 more of 68 total
- South Australian Scleroderma Register: autoantibodies as predictive biomarkers of phenotype and outcome.
2012 · Int J Rheum Dis · RCR 2.8 · 82 citations - A small nucleolar RNA is processed from an intron of the human gene encoding ribosomal protein S3.
1993 · Genes Dev · RCR 2.8 · 167 citations - Anti-fibrillarin antibodies in systemic sclerosis.
2001 · Rheumatology (Oxford) · RCR 2.5 · 92 citations - Mercury exposure, malaria, and serum antinuclear/antinucleolar antibodies in Amazon populations in Brazil: a cross-sectional study.
2004 · Environ Health · RCR 2.4 · 77 citations - The autoimmunity-inducing xenobiotic mercury interacts with the autoantigen fibrillarin and modifies its molecular and antigenic properties.
1997 · J Immunol · RCR 2.4 · 98 citations - Adverse immunological effects and autoimmunity induced by dental amalgam and alloy in mice.
1994 · FASEB J · RCR 2.3 · 59 citations - Mercury-induced autoimmunity in mice.
2002 · Environ Health Perspect · RCR 2.2 · 72 citations - Analysis of the autoantibody response to fibrillarin in human disease and murine models of autoimmunity.
1995 · J Immunol · RCR 1.9 · 73 citations - Dynamic localization of RNase MRP RNA in the nucleolus observed by fluorescent RNA cytochemistry in living cells.
1995 · J Cell Biol · RCR 1.8 · 96 citations - Murine genotype influences the specificity, magnitude and persistence of murine mercury-induced autoimmunity.
1996 · J Autoimmun · RCR 1.8 · 59 citations - Autoantibodies in systemic sclerosis and fibrosing syndromes: clinical indications and relevance.
2004 · Curr Opin Rheumatol · RCR 1.6 · 57 citations - Autoreactive B cell responses targeting nuclear antigens in systemic sclerosis: Implications for disease pathogenesis.
2023 · Semin Arthritis Rheum · RCR 1.4 · 11 citations - The effect of dose, gender, and non-H-2 genes in murine mercury-induced autoimmunity.
2001 · J Autoimmun · RCR 1.4 · 44 citations - Selective induction of anti-fibrillarin autoantibodies by silver nitrate in mice.
1994 · Clin Exp Immunol · RCR 1.4 · 47 citations - Antifibrillarin Antibodies Are Associated with Native North American Ethnicity and Poorer Survival in Systemic Sclerosis.
2017 · J Rheumatol · RCR 1.3 · 27 citations - Anti-fibrillarin antibody in African American patients with systemic sclerosis: immunogenetics, clinical features, and survival analysis.
2011 · J Rheumatol · RCR 1.3 · 41 citations - Autoantibodies to ribosomal P antigens with immune complex glomerulonephritis in SJL mice treated with pristane.
1996 · J Immunol · RCR 1.3 · 51 citations - The Clinical Relevance of Antifibrillarin (anti-U3-RNP) Autoantibodies in Systemic Sclerosis.
2017 · Scand J Immunol · RCR 1.3 · 28 citations - The immunosuppressive effect of methylmercury does not preclude development of autoimmunity in genetically susceptible mice.
2005 · Toxicology · RCR 1.3 · 37 citations - Autoantibodies against B23, a nucleolar phosphoprotein, occur in scleroderma and are associated with pulmonary hypertension.
2003 · Arthritis Rheum · RCR 1.2 · 53 citations
Reference: B cellIEDB
1 publication
- Computational analysis of high-density peptide microarray data with application from systemic sclerosis to multiple sclerosis.
2012 · Autoimmun Rev · RCR 1 · 35 citations
Sources: PubMed — antigen-level antibody evidence from a custom retrieval. Records matching a controlled set of autoantibody terms (the MeSH descriptors Autoantibodies and Autoantigens, with title and abstract term variants) were obtained through NCBI E-utilities, and their titles and abstracts parsed for constructions that direct an antibody at a named protein rather than for co-occurrence. Captured names were resolved against UniProt nomenclature and each antigen adjudicated individually against the source text. IEDB — curated epitope assays from the Immune Epitope Database (Vita et al., Nucleic Acids Research 2019). Bibliographic records from PubMed and MeSH, U.S. National Library of Medicine; citation metrics from NIH iCite (Hutchins et al., PLoS Biology 2016). Titles link to PubMed; abstracts are not reproduced here. The NLM does not endorse this analysis.
Genetic constraint and essentialitygnomAD · DepMap
Does the body need this protein intact? Low LOEUF or a strong DepMap dependency means loss or blockade of the protein is likely to be felt.
- gnomAD LOEUF (loss-of-function intolerance)
- 0.25
- gnomAD pLI
- 0.99
- gnomAD missense Z
- 1.2
- DepMap mean gene effect
- -1.13
- DepMap dependency class
- common
Cancer expressionTCGA
Across TCGA tumor cohorts, this protein is over-expressed in roughly 8% of surveyed tumor types (aggregate summary; per-cohort expression, alteration, and survival load in the interactive view).
OntologyGO
Biological processes
- box C/D sno(s)RNA 3'-end processing
- osteoblast differentiation
- ribosomal small subunit biogenesis
- rRNA methylation
- rRNA processing
- snoRNA localization
Molecular functions
- ATPase binding
- histone H2AQ104 methyltransferase activity
- RNA binding
- rRNA methyltransferase activity
- TFIID-class transcription factor complex binding
- U6 snRNA 2'-O-ribose methyltransferase activity
Cellular components
Protein domainsUniProt · Pfam · InterPro
KeywordsUniProt
InteractionsUniProt · HPA
Protein binding partners of FBL in the human serome: UniProt-annotated complex subunits plus reported interactors. Each links to its own Seroatlas record.
Antibody and autoantibody relevanceSeroatlas analysis
Seroatlas reads FBL as an antibody target. Whether an autoantibody or antibody against FBL could matter depends on whether native FBL is physically reachable, whether the body needs it intact, and whether it acts in a disease-relevant tissue.
FBL is annotated as predominantly intracellular. Intracellular proteins are common autoantibody markers, becoming visible to the immune system after cell injury or altered processing, but are usually markers of disease rather than direct drivers.
Source-annotated serology context
The source annotations explicitly mention antibody, autoantibody, autoantigen, or autoimmune context. This is biological context, not study-specific reactivity.
- Antisera from approximately 8% of humans with the autoimmune disease scleroderma recognize fibrillarin.
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