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FLASH GENE
Symbol FBL contributors: mct - updated : 27-08-2014
HGNC name fibrillarin
HGNC id 3599
Location 19q13.2      Physical location : 40.325.093 - 40.337.054
Synonym name
  • 34 kDa nucleolar scleroderma antigen
  • rRNA 2'-O-methyltransferase fibrillarin
  • RNA, U3 small nucleolar interacting protein 1
  • Synonym symbol(s) FLRN, FIB, RNU3IP1, FIB1
    EC.number 2.1.1.-
    DNA
    TYPE functioning gene
    STRUCTURE 11.96 kb     9 Exon(s)
    10 Kb 5' upstream gene genomic sequence study
    MAPPING cloned Y linked N status provisional
    Physical map
    IL28A 19q13.13 interleukin 28A (interferon, lambda 2) IL29 19q13.13 interleukin 29 (interferon, lambda 1) KIAA1484 GMFG 19p13.2-p12 glia maturation factor, gamma FLJ10211 19q13.2 hypothetical protein FLJ10211 PD2 19q13.1 hypothetical protein F23149_1 Intersex 19q13.2 homolog of Drosophila Intersex ZFP36 19q13.1 zinc finger protein 36, C3H type, homolog (mouse) CLG 19q13.2 likely ortholog of mouse common-site lymphoma/leukemia GEF RPS16 19q13.1 ribosomal protein S16 SUPT5H 19q13 suppressor of Ty 5 homolog (S. cerevisiae) TIMM50 19q13.2 translocase of inner mitochondrial membrane 50 homolog (yeast) DLL3 19q13.1-13.3 delta-like 3 (Drosophila) SELV 19q13.2 selenoprotein V FLJ38944 19q13.2 hypothetical protein FLJ38944 MGC20452 LOC390930 19 similar to Charot-Leyden crystal protein; Charcot-Leyden crystal protein; eosinophil lysophospholipase; lysolecithin acylhydrolase; galactin-10 PP13 19q13.1 placental protein 13 LOC390931 19 similar to placental protein 13-like protein LOC148003 19q13.2 similar to placental protein 13; galectin-13 LOC390932 19 similar to Eosinophil lysophospholipase (Charcot-Leyden crystal protein homolog) (Lysolecithin acylhydrolase) (CLC) (Galectin-10) PPL13 19q13.2 placental protein 13-like protein CLC 19q13.1 Charot-Leyden crystal protein LOC342900 19q13.2 hypothetical LOC342900 DYRK1B 19q12-q13.11 dual-specificity tyrosine-(Y)-phosphorylation regulated kinase 1B FBL 19q13.1 fibrillarin FCGBP 19q13.1 Fc fragment of IgG binding protein LOC388540 19 hypothetical gene supported by BX640962; NM_018457 PSMC4 19q13.11-q13.13 proteasome (prosome, macropain) 26S subunit, ATPase, 4 MGC43537 19q13.2 hypothetical protein MGC43537 LOC390933 19 similar to hypothetical protein FLJ36874 LOC163131 19q13.2 similar to BC331191_1 LOC284323 19q13.2 hypothetical protein LOC284323 MAP3K10 19q13.2 mitogen-activated protein kinase kinase kinase 10 FLJ30373 19q13.2 hypothetical protein FLJ30373 FLJ13265 19q13.2 hypothetical protein FLJ13265 AKT2 19q13.2 v-akt murine thymoma viral oncogene homolog 2 FLJ36888 19q13.2 hypothetical protein FLJ36888
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    9 - 1171 33 321 - Amin (2007)
    EXPRESSION
    Type ubiquitous
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Endocrinepancreas   highly
    Reproductivefemale systemovary  highly
     female systemuteruscervix highly
    Visualeye    
    cell lineage
    cell lines
    fluid/secretion
    at STAGE
    PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    motifs/domains
  • two cysteine residues (Cys99 and Cys268) whose sulfhydryl groups are able to establish intramolecular -S-S- bridges
  • HOMOLOGY
    interspecies homolog to yeast S.cerevisiae NOP1
    ortholog to yeast S.cerevisiae
    ortholog to xenopus nop1
    Homologene
    FAMILY
    CATEGORY RNA associated
    SUBCELLULAR LOCALIZATION     plasma membrane
        intracellular
    intracellular,cytoplasm,organelle,endosome
    intracellular,nucleus,chromatin/chromosome
    intracellular,nucleus,nucleolus
    text
  • fibrillar region of the nucleolus but also Cajal bodies
  • pre-rRNA processing factors such as DKC1, NOLC1 and fibrillarin (FBL) associate with r-chromatin during interphase but were released from r-chromatin in mitosis
  • basic FUNCTION
  • evolutionarily-conserved and obligatory protein component of eukaryotic cell nucleoli involved in pre-rRNA processing and methylation
  • its form without disulfide bridges is included into the mobile protein fraction and is consistent with its functionally active state
  • FBL and RRP1 are associated with pre-ribosome particles distinctly different from each other
  • its overexpression contributes to tumorigenesis and is associated with poor survival in patients with breast cancer
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
    INTERACTION
    DNA
    RNA
    small molecule
    protein
  • by deubiquitylating various nucleolar substrate proteins including NPM1 and FBL, USP36 plays a crucial role in regulating the structure and function of nucleoli
  • FBL and NOP56 interact before being co-assembled in box C/D snoRNPs
  • is a USP36 substrate protein in nucleoli (pMID: 19679658)
  • the presence of CBX1 in nucleoli is likely connected with transcription of ribosomal genes and with the role of fibrillarin in nucleolar processes
  • FBL and and NOP56 directly interact (interaction, indispensable for the association of both proteins with the box C/D snoRNPs)
  • C1QBP is a new rRNA maturation factor involved in the remodeling from pre-90S particles to pre-40S and pre-60S particles that requires the exchange of FBL for RRP1
  • TP53 represses expression of the rRNA methyl-transferase fibrillarin (FBL) by binding directly to FBL
  • rRNA gene chromatin (r-chromatin) association of NOLC1, DKC1, and FBL was dependent on the transcription factor upstream binding factor (UBF)
  • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s)
    Susceptibility to systemic sclerosis
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS