WRNIP1

Werner's syndrome is a rare autosomal recessive disorder characterized by accelerated aging that is caused by defects in the Werner syndrome ATP-dependent helicase gene (WRN). The protein encoded by this gene interacts with the exonuclease-containing N-terminal portion of the Werner protein. This protein has a ubiquitin-binding zinc-finger domain in the N-terminus, an ATPase domain, and two leucine zipper motifs in the C-terminus. It has sequence similarity to replication factor C family proteins and is conserved from E. coli to human. This protein likely accumulates at sites of DNA damage by interacting with polyubiquinated proteins and also binds to DNA polymerase delta and increases the initiation frequency of DNA polymerase delta-mediated DNA synthesis. This protein also interacts with nucleoporins at nuclear pore complexes. Two transcript variants encoding different isoforms have been isolated for this gene.

Full Name

Werner helicase interacting protein 1

Function

Functions as a modulator of initiation or reinitiation events during DNA polymerase delta-mediated DNA synthesis. In the presence of ATP, stimulation of DNA polymerase delta-mediated DNA synthesis is decreased. Also plays a role in the innate immune defense against viruses. Stabilizes the RIGI dsRNA interaction and promotes RIGI 'Lys-63'-linked polyubiquitination. In turn, RIGI transmits the signal through mitochondrial MAVS.

Biological Process

Biological Process DNA synthesis involved in DNA repair Source:UniProtKB1 Publication
Biological Process DNA-templated DNA replication Source:GO_Central1 Publication
Biological Process innate immune response Source:UniProtKB-KW
Biological Process regulation of DNA repair Source:GO_Central1 Publication
Biological Process regulation of DNA-templated DNA replication initiation Source:UniProtKB1 Publication

Cellular Location

Nucleus
Cytoplasm
Colocalizes with WRN in granular structures in the nucleus.

PTM

Sumoylated with SUMO1 and SUMO2/3.