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FLASH GENE
Symbol FBL contributors: mct - updated : 27-08-2014
HGNC name fibrillarin
HGNC id 3599
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