Werner Syndrome Helicase (Phospho-Ser1141) (WRN) Antibody – Rabbit

This gene encodes a member of the RecQ subfamily and the DEAH (Asp-Glu-Ala-His) subfamily of DNA and RNA helicases. DNA helicases are involved in many aspects of DNA metabolism, including transcription, replication, recombination, and repair. This protein contains a nuclear localization signal in the C-terminus and shows a predominant nucleolar localization. It possesses an intrinsic 3' to 5' DNA helicase activity, and is also a 3' to 5' exonuclease. Based on interactions between this protein and Ku70/80 heterodimer in DNA end processing, this protein may be involved in the repair of double strand DNA breaks. Defects in this gene are the cause of Werner syndrome, an autosomal recessive disorder characterized by premature aging. [provided by RefSeq, Jul 2008]

WRN: Multifunctional enzyme that has both magnesium and ATP- dependent DNA-helicase activity and 3'->5' exonuclease activity towards double-stranded DNA with a 5'-overhang. Has no nuclease activity towards single-stranded DNA or blunt-ended double- stranded DNA. Binds preferentially to DNA substrates containing alternate secondary structures, such as replication forks and Holliday junctions. May play an important role in the dissociation of joint DNA molecules that can arise as products of homologous recombination, at stalled replication forks or during DNA repair. Alleviates stalling of DNA polymerases at the site of DNA lesions. Important for genomic integrity. Plays a role in the formation of DNA replication focal centers; stably associates with foci elements generating binding sites for RP-A. Monomer, and homooligomer. May exist as homodimer, homotrimer, homotetramer and/or homohexamer. Homotetramer, or homohexamer, when bound to DNA. Interacts via its N-terminal domain with WRNIP1. Interacts with EXO1, PCNA and SUPV3L1. Belongs to the helicase family. RecQ subfamily.

Protein type: Nucleolus; EC 3.6.4.12; DNA repair, damage; Helicase; DNA-binding; Cell cycle regulation

Chromosomal Location of Human Ortholog: 8p12

Cellular Component: nucleoplasm; MutLalpha complex; centrosome; nucleolus

Molecular Function: G-quadruplex DNA binding; protein homodimerization activity; ATPase activity; magnesium ion binding; 3'-5' DNA helicase activity; bubble DNA binding; helicase activity; four-way junction helicase activity; ATP-dependent DNA helicase activity; Y-form DNA binding; protein binding; DNA helicase activity; DNA binding; ATP-dependent 3'-5' DNA helicase activity; manganese ion binding; exonuclease activity; protein complex binding; 3'-5' exonuclease activity; ATP binding

Biological Process: nucleolus to nucleoplasm transport; positive regulation of hydrolase activity; multicellular organismal aging; cell aging; cellular response to starvation; replicative cell aging; response to UV-C; DNA duplex unwinding; replication fork processing; DNA recombination; regulation of apoptosis; DNA synthesis during DNA repair; base-excision repair; double-strand break repair; regulation of growth rate; response to oxidative stress; DNA replication; response to DNA damage stimulus; DNA metabolic process; telomere maintenance; aging

Disease: Werner Syndrome