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FLASH GENE
Symbol PRPF19 contributors: mct - updated : 24-05-2023
HGNC name PRP19/PSO4 pre-mRNA processing factor 19 homolog (S. cerevisiae)
HGNC id 17896
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • a domain of AAs 167-250 is necessary for the recruitment of PRPF19 to lipid droplets and a domain of AAs 1-166 is necessary for the recruitment of PRPF19 to a nucleus
  • C-terminal WD40 repeat domain, involved in variety of processes in eukaryotic cells
  • secondary structure WD40 domain with a canonical seven-bladed beta-propeller fold, consisting of seven WD40 repeats
    HOMOLOGY
    interspecies ortholog to yeast Prp19
    Homologene
    FAMILY
    CATEGORY enzyme
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,cytosolic
    intracellular,cytoplasm,cytoskeleton,microtubule,mitotic spindle
    intracellular,nucleus,nucleoplasm,nuclear bodies,nuclear speckles
    text
  • found in the nucleus, cytosol or lipid droplets depending on the cell type
  • basic FUNCTION
  • playing a role in pre-mRNA splicing, but also having a direct role in DNA damage processing
  • extending the cellular life span by increasing the resistance to stress or by improving the DNA repair capacity of the cells
  • nuclear matrix protein involved in pre-mRNA splicing, ubiquitylation, and DNA repair
  • involved in pre-mRNA splicing, having an essential role for spliceosome assembly and spliceosome stability
  • is necessary to bring SETMAR to the DSB sites for its function(s) in DNA repair
  • once it forms a complex with SETMAR, negatively regulates its TIR binding activity, which is perhaps necessary for SETMAR localization at non-TIR sites such as double-strand breaks
  • spliceosomal PRPF19 complex modifies PRPF3, a component of the USP4, with nonproteolytic K63-linked ubiquitin chains
  • multi-functional protein that plays a role in splicing, ubiquitination, and cell growth
  • both U2AF2 and PRPF19 are required for RNA polymerase II C-terminal domain-dependent splicing activation
  • PRPF19 splicing complex is potentially a novel transcription elongation factor
  • involved in cellular life-span regulation and proteasomal breakdown
  • PRPF19 participated in mitotic progression in hepatocellular carcinoma (HCC)
  • PRPF19 plays an important role in the induction of TP53-dependent cellular senescence 7)
  • PRPF19 promotes cell viability and slows down the aging process in human skin
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
  • PRPF19 and its related proteins are major components of the spliceosome catalytic core RNP
  • PRPF19/CDC5L complex contains four copies of PRPF19
  • important component in human spliceosome machinery
  • INTERACTION
    DNA
    RNA
    small molecule
    protein
  • interact with WRN through a direct physical association between WRN and CDC5L
  • SETMAR binding partner that mediates SETMAR binding to non-TIR DNA such as DNA damage sites
  • is involved in DNA repair and splicing, associates with the proteasome by directly binding to the beta7 subunit of the 20 S proteasome
  • CTNNBL1 associates with the PRPF19 complex of the spliceosome, interacting with its CDC5L component
  • BCAS2 directly interacts with each component of the PRPF19/CDC5L complex core
  • interacts with PHD3 via its C-terminal WD40 region, and the interaction is enhanced under hypoxic conditions through the utilization of the N-terminal coiled-coil domain
  • CTNNBL1 associated with PRPF19-containing RNA-splicing complexes as well as with the antibody-diversifying enzyme AICDA
  • SNW1-associated factors DHX8 and PRPF19 are also selectively required for CDKN1A expression under stress
  • EXOC7 interacts directly with PRPF19 and shuttles to the nucleus, where it associates with the spliceosome
  • DHX15 and PRPF19, two spliceosome components, are novel RBM5-interacting partner
  • CCDC94 and the PRPF9 complex protect cells from IR-induced apoptosis by repressing the expression of TP53 mRNA
  • PRPF19 complex is the first spliceosome subcomplex that directly contributes to mitosis in vertebrates independently of its function in interphase
  • BCAS2 and PRPF19 subunits of the PSO4 complex directly interact and colocalize with replication protein A (RPA1)
  • PRPF19 interacts with CDC5L directly
  • PRPF19 downregulation inhibits MDM4-mediated TP53 inactivation, resulting in induction of cellular senescence 7)
  • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s)
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    constitutional     --low  
    in various cell types during replicative senescence
    tumoral     --over  
    in various immortalized cell lines, as well as in breast tumors, with good survival
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    SystemTypeDisorderPubmed
    cancerdigestiveliver
    could be a promising therapeutic target of HCC
    ANIMAL & CELL MODELS