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FLASH GENE
Symbol UPF3B contributors: mct - updated : 03-10-2017
HGNC name UPF3 regulator of nonsense transcripts homolog B (yeast)
HGNC id 20439
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • one RNA recognition motif (RRM)
  • Smg4/UPF3 domain
    • HOMOLOGY
    interspecies ortholog to yeast S.cerevisiae Upf3p
    homolog to Drosophila DS07851.3
    ortholog to C.elegans C23G10.2/SMG.4
    intraspecies paralog to UPF3A
    Homologene
    FAMILY nonsense-mediated mRNA decay complex
    CATEGORY RNA associated
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm
    intracellular,nucleus,nucleolus
    text
  • in neural stem cells, UPF3B protein was detected in the cytoplasm and in the nucleus, and enriched in the nucleolus
    • basic FUNCTION
  • involved in both mRNA nuclear export and mRNA surveillance for the detection of truncated messengers and initiation of nonsense mediated mRNA decay NMD
  • important component of the nonsense-mediated mRNA decay surveillance machinery
  • acting as a nucleocytoplasmic shuttle
  • UPF3A levels are tightly regulated by a post-transcriptional switch to maintain appropriate levels of NMD substrates in cells containing different levels of UPF3B
  • is a core member of the nonsense-mediated mRNA decay (NMD) pathway that functions to rapidly degrade transcripts with premature termination codons (PTCs)
  • MYCN, PIGV and UPF3B regulate likely synapse development
  • is involved in the nonsense-mediated mRNA decay pathway (NMD) that controls mRNA stability and functions in the prevention of the synthesis of truncated proteins
  • UPF3B and UPF1 are down-regulated during differentiation of neural stem cells into neurons
  • UPF3B--is critical for the nonsense-mediated RNA decay (NMD) pathway, while its autosomal counterpart--UPF3A--encodes an enigmatic protein previously shown to have trace NMD activity
  • interacts with the release factors, delays translation termination and dissociates post-termination ribosomal complexes that are devoid of the nascent peptide
  • is likely involved in the crosstalk between the NMD machinery and the premature termination codons (PTCs)-bound ribosome, a central mechanistic step of RNA surveillance
    • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component component of a post-splicing multiprotein complex
    INTERACTION
    DNA
    RNA binding specifically to 20nt upstream of exon-exon junctions in mRNAs
    small molecule
    protein
  • RBM8A, THOC4 (ALY), NXF1 (TAP), UPF2
  • DCP2
  • in the UPF trimeric complex, UPF2 and UPF3B cooperatively stimulate both ATPase and RNA helicase activities of UPF1
  • SATB2 binds to the UPF3B promoter, and SATB2 expression significantly activates gene transcription using the UPF3B promoter
  • ability of UPF1 to impinge on premature termination, moreover, requires ATP-binding, RNA-binding and NMD cofactors UPF2 and UPF3A, UPF3B
  • UPF1 and ribosomes are new interaction partners of UPF3B
    • cell & other
    REGULATION
    activated by SATB2, that activates UPF3B expression through binding to its promoter
    ASSOCIATED DISORDERS
    corresponding disease(s) LJFS3 , FGS6 , MRXS14
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS