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FLASH GENE
Symbol HNRNPU contributors: mct - updated : 14-02-2018
HGNC name heterogeneous nuclear ribonucleoprotein U (scaffold attachment factor A)
HGNC id 5048
PROTEIN
PHYSICAL PROPERTIES acid
STRUCTURE
motifs/domains
  • ASP/GLU (acidic) rich domain
  • SAP domain
  • a poly Glu domain
  • Gln rich domain
  • ATP binding domain
  • Gly rich domain with RNA binding domain( RGG-box) and poly Gly domain
  • nuclear localization signal
  • The C-terminal 187-AAs domain was necessary and sufficient for the association with both TOP2B and the endogenous RNA
    • conjugated RiboP , PhosphoP
    mono polymer complex
    HOMOLOGY
    interspecies ortholog to murine Hnrpu
    homolog to rattus Hnrpu
    homolog to Xenopus Xl.8951
    homolog to C.elegans Y41E3.11
    Homologene
    FAMILY
    CATEGORY DNA associated , RNA associated
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,cytoskeleton,microtubule,centrosome
    intracellular,cytoplasm,cytoskeleton,microtubule,mitotic spindle
    intracellular,nucleus,nucleoplasm,nuclear bodies,nuclear speckles
    intracellular,nucleus,chromatin/chromosome,nucleosome
    intracellular,nucleus,chromatin/chromosome,kinetochore
    text
  • during mitosis, localized at the spindles, spindle midzone and cytoplasmic bridge
  • colocalized with TPX2 and AURKA in spindle poles and Microtubules
    • basic FUNCTION
  • involved RNA processing
  • abundant nuclear protein that interacts specifically with nuclear matrix attachment region DNA (MAR) and RNA as a component of hnRNPs
  • contributes to the attachment of spindle microtubules (MTs) to kinetochores and spindle organization
  • is a novel spindle regulator that plays an essential role in kinetochore-Microtubule attachment and mitotic spindle organization
  • implicated in diverse pathways from transcriptional regulation to telomere length control to X inactivation
  • potent regulator of nuclear ribonucleoprotein particles in diverse gene expression pathways
  • HNRNPU is essential for H19-mediated transcription repression
  • essential role of HNRNU in heart development and function and in the regulation of alternative splicing
  • contributes to stabilize the kinetochore-microtubule interaction during mitosis
    • CELLULAR PROCESS cell life, cell death/apoptosis
    nucleotide, chromatin organization
    protein, post translation, targeting
    cell organization/biogenesis
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
    INTERACTION
    DNA binding to double stranded and single stranded DNA
    high affinity for scaffold attached-region (SAR) DNA
    RNA binding to pre-mRNA
    small molecule nucleotide,
  • ATP
    • protein
  • binding to caspase 3, BTRC, p300
  • ASH2L is recruited by XIST concomitantly with HNRNPU and macroH2A at the transition to inactive X chromosome maintenance
  • TOP2B associates stoichiometrically with HNRNPU in the presence of RNA
  • interacts with the C-terminal domain (CTD) of endogenous RNA polymerase II and the interaction is independent of CTD phosphorylation and mRNA
  • can bind to MTs (Microtubules) and contributes to the targeting of AURKA to mitotic spindle MTs
  • HNRNPU interacts with CENPW, and the interaction between HNRNPU and CENPW mutually increased each other's protein stability by inhibiting the proteasome-mediated degradation
  • originally identified as a structural nuclear protein, interacts with chromatin-associated RNAs (caRNAs) via its RGG domain to regulate interphase chromatin structures in a transcription-dependent manner
    • cell & other
    REGULATION
    Phosphorylated by PRKDC (phosphorylated at Ser59 by PRKDC in cells in response to DNA double-strand breaks and phosphorylation of HNRNPU suggests novel functions for PRKDC in the response to DNA damage)
    ASSOCIATED DISORDERS
    corresponding disease(s) DEL1Q44 , EIEE54
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS
  • mice lacking the heterogeneous nuclear ribonucleoprotein U (hnRNPU) in the heart develop lethal dilated cardiomyopathy and display numerous defects in cardiac pre-mRNA splicing