Function: Plays a role in DNA double-strand break (DSB) repair. Binds double-stranded DNA in a sequence-nonspecific manner. Acts as a structural component of the nuclear framework. May also serve as a support for spliceosome binding and activity. Essential for spliceosome assembly in a oligomerization-dependent manner and might also be important for spliceosome stability. May have E3 ubiquitin ligase activity. The PSO4 complex is required in the DNA interstrand cross-links (ICLs) repair process. Component of the PRP19-CDC5L complex that forms an integral part of the spliceosome and is required for activating pre-mRNA splicing. Ref.1 Ref.2 Ref.9 Ref.10 Ref.11 Ref.12
Subunit structure: Homooligomer. Identified in the spliceosome C complex. Component of the PSO4 complex, composed of PRPF19, CDC5L, PLRG1. Component of the PRP19-CDC5L splicing complex composed of a core complex comprising a homotetramer of PRPF19, CDC5L, PLRG1 and BCAS2, and at least three less stably associated proteins CTNNBL1, CWC15 and HSPA8. Interacts in the complex directly with CDC5L, PLRG1 and BCAS2. Interacts with APEX1, DNTT and PSMB4. Interacts with CWC22 and EIF4A3 in an RNA-independent manner. Ref.2 Ref.8 Ref.9 Ref.10 Ref.11 Ref.15 Ref.17 Ref.19
Subcellular location: Nucleus. Nucleus › nucleoplasm. Cytoplasm › cytoskeleton › spindle. Note: Nucleoplasmic in interphase cells. Irregularly distributed in anaphase cells. In prophase cells, uniformly distributed, but not associated with condensing chromosomes. Found in extrachromosomal regions in metaphase cells. Mainly localized to the mitotic spindle apparatus when chromosomes segregate during anaphase. When nuclei reform during late telophase, uniformly distributed in daughter cells and displays no preferred association with decondensing chromatin. Ref.1 Ref.7 Ref.15 Ref.17
Tissue specificity: Ubiquitous. Weakly expressed in senescent cells of different tissue origins. Highly expressed in tumor cell lines. Ref.1 Ref.2 Ref.6 Ref.12
Induction: By gamma irradiation and chemical mutagens but not by UV irradiation. Ref.2
Sequence similarities: Belongs to the WD repeat PRP19 family.Contains 1 U-box domain.Contains 7 WD repeats.
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