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

Function: 1) role in DNA double-strand break repair & pre-mRNA splicing 2) binds double-stranded DNA in a sequence-nonspecific manner 3) structural component of the nuclear framework 4) support for spliceosome binding & activity 5) essential for spliceosome assembly (oligomerization-dependent) 6) spliceosome stability 7) E3 ubiquitin ligase activity (putative) 8) component of PSO4 complex is required in the DNA interstrand cross-link repair process 9) component of spliceosome C complex 10) interacts with DNTT/TdT & PSMB4 11) 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

General

DNA-binding protein ligase or synthetase WD repeat protein family

Properties

SIZE: MW = 55 kD entity length = 504 aa COMPARTMENT: cell nucleus MOTIF: U-box {1-68} WD repeat {219-259} WD repeat {262-301} WD repeat {304-345} WD repeat {348-387} WD repeat {390-429} WD repeat {433-472} WD repeat {473-503}

Database Correlations

OMIM 608330 UniProt Q9UMS4 PFAM correlations

References

UniProt :accession Q9UMS4