scholarly journals The Werner syndrome protein: linking the replication checkpoint response to genome stability

Aging ◽  
2011 ◽  
Vol 3 (3) ◽  
pp. 311-318 ◽  
Author(s):  
Pietro Pichierri ◽  
Francesca Ammazzalorso ◽  
Margherita Bignami ◽  
Annapaola Franchitto
Keyword(s):  
Genome Stability ◽  
Werner Syndrome ◽  
Replication Checkpoint ◽  
Checkpoint Response ◽  
Werner Syndrome Protein ◽  
Syndrome Protein

scholarly journals Werner Syndrome Protein and DNA Replication

2018 ◽  
Vol 19 (11) ◽  
pp. 3442 ◽  
Author(s):  
Shibani Mukherjee ◽  
Debapriya Sinha ◽  
Souparno Bhattacharya ◽  
Kalayarasan Srinivasan ◽  
Salim Abdisalaam ◽  
...  
Keyword(s):  
Dna Replication ◽  
Ataxia Telangiectasia ◽  
Replication Fork ◽  
Genome Stability ◽  
Werner Syndrome ◽  
Genotoxic Stress ◽  
Premature Aging ◽  
Replication Forks ◽  
Werner Syndrome Protein ◽  
Syndrome Protein

Werner Syndrome (WS) is an autosomal recessive disorder characterized by the premature development of aging features. Individuals with WS also have a greater predisposition to rare cancers that are mesenchymal in origin. Werner Syndrome Protein (WRN), the protein mutated in WS, is unique among RecQ family proteins in that it possesses exonuclease and 3′ to 5′ helicase activities. WRN forms dynamic sub-complexes with different factors involved in DNA replication, recombination and repair. WRN binding partners either facilitate its DNA metabolic activities or utilize it to execute their specific functions. Furthermore, WRN is phosphorylated by multiple kinases, including Ataxia telangiectasia mutated, Ataxia telangiectasia and Rad3 related, c-Abl, Cyclin-dependent kinase 1 and DNA-dependent protein kinase catalytic subunit, in response to genotoxic stress. These post-translational modifications are critical for WRN to function properly in DNA repair, replication and recombination. Accumulating evidence suggests that WRN plays a crucial role in one or more genome stability maintenance pathways, through which it suppresses cancer and premature aging. Among its many functions, WRN helps in replication fork progression, facilitates the repair of stalled replication forks and DNA double-strand breaks associated with replication forks, and blocks nuclease-mediated excessive processing of replication forks. In this review, we specifically focus on human WRN’s contribution to replication fork processing for maintaining genome stability and suppressing premature aging. Understanding WRN’s molecular role in timely and faithful DNA replication will further advance our understanding of the pathophysiology of WS.

scholarly journals Identification of a Coiled Coil in Werner Syndrome Protein That Facilitates Multimerization and Promotes Exonuclease Processivity

2010 ◽  
Vol 285 (33) ◽  
pp. 25699-25707 ◽  
Author(s):  
J. Jefferson P. Perry ◽  
Aroumougame Asaithamby ◽  
Adam Barnebey ◽  
Foad Kiamanesch ◽  
David J. Chen ◽  
...  
Keyword(s):  
Werner Syndrome ◽  
Coiled Coil ◽  
Werner Syndrome Protein ◽  
Syndrome Protein

Analyses of the interaction of WRNIP1 with Werner syndrome protein (WRN) in vitro and in the cell

DNA Repair ◽  
2006 ◽  
Vol 5 (7) ◽  
pp. 816-828 ◽  
Author(s):  
Yoh-ichi Kawabe ◽  
Masayuki Seki ◽  
Akari Yoshimura ◽  
Katsuaki Nishino ◽  
Tomoko Hayashi ◽  
...  
Keyword(s):  
Werner Syndrome ◽  
Werner Syndrome Protein ◽  
Syndrome Protein

scholarly journals Probing the Roles of Active Site Residues in the 3′-5′ Exonuclease of the Werner Syndrome Protein

2007 ◽  
Vol 282 (13) ◽  
pp. 9941-9951 ◽  
Author(s):  
Jung Min Choi ◽  
Sung Yun Kang ◽  
Won Jin Bae ◽  
Kyeong Sik Jin ◽  
Moonhor Ree ◽  
...  
Keyword(s):  
Active Site ◽  
Werner Syndrome ◽  
Active Site Residues ◽  
Werner Syndrome Protein ◽  
Syndrome Protein

scholarly journals Serines 440 and 467 in the Werner syndrome protein are phosphorylated by DNA-PK and affects its dynamics in response to DNA double strand breaks

Aging ◽  
2014 ◽  
Vol 6 (1) ◽  
pp. 70-81 ◽  
Author(s):  
Rika Kusumoto-Matsuo ◽  
Deblina Ghosh ◽  
Parimal Karmakar ◽  
Alfred May ◽  
Dale Ramsden ◽  
...  
Keyword(s):  
Werner Syndrome ◽  
Double Strand Breaks ◽  
Dna Double Strand Breaks ◽  
Strand Breaks ◽  
Werner Syndrome Protein ◽  
Syndrome Protein
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