pre-mRNA-processing factor 19 (PRP19/PSO4 homolog, hPso4, nuclear matrix protein 200, senescence evasion factor, PRPF19, NMP200, PRP19, SNEV)

Function

• role in DNA double-strand break repair & pre-mRNA splicing
• binds double-stranded DNA in a sequence-nonspecific manner
• structural component of the nuclear framework
• support for spliceosome binding & activity
• essential for spliceosome assembly (oligomerization-dependent)
• spliceosome stability
• E3 ubiquitin ligase activity (putative)
• component of PSO4 complex is required in the DNA interstrand cross-link repair process
• component of spliceosome C complex
• interacts with DNTT/TdT & PSMB4
• overexpression of PRPF19 might extend the cellular life span by increasing the resistance to stress or by improving DNA repair capacity of cells

Structure

• homooligomer
• contains 1 U-box domain
• contains 7 WD repeats

Compartment

• nucleus, nucleoplasm
• 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 when chromosomes segregate during anaphase
• when nuclei reform during late telophase, uniformly distributed in daughter cells & displays no preferred association with decondensing chromatin

Expression

• ubiquitous
• weakly expressed in senescent cells of different tissue
• induced by gamma radiation & chemical mutagens but not by UV

Pathology

• highly expressed in tumor cell lines

More general terms

• ligase or synthetase
• WD repeat protein family
• DNA-binding protein

Component of

• spliceosome C complex

Database

• OMIM: https://mirror.omim.org/entry/608330
• UniProt: http://www.uniprot.org/uniprot/Q9UMS4.html