The Werner syndrome protein is a DNA helicase

@article{Gray1997TheWS,
  title={The Werner syndrome protein is a DNA helicase},
  author={Matthew D. Gray and Jiang-Cheng Shen and Ashwini S. Kamath-Loeb and A. Blank and Bryce L. Sopher and George M Martin and Junko Oshima and Lawrence A. Loeb},
  journal={Nature Genetics},
  year={1997},
  volume={17},
  pages={100-103}
}
Werner syndrome (WS) is an uncommon autosomal recessive disorder characterized by premature aging. The clinical manifestations of WS, including atherosclerosis and osteoporosis, appear early in adulthood, and death in the fourth to sixth decade commonly ensues from myocardial infarction or cancer1,2. In accord with the aging phenotype, cells from WS patients have a reduced replicative life span in culture3. Genomic instability is observed at the cytogenetic level in the form of chromosome… 
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Unwinding the molecular basis of the Werner syndrome
The Werner syndrome helicase-nuclease--one protein, many mysteries.
  • M. Fry
  • Biology
    Science of aging knowledge environment : SAGE KE
  • 2002
TLDR
The biochemistry of the WRN protein and some aspects of its cell biology are focused on, including the putative functions of WRN in normal cells and the consequences of the loss of these functions in WS.
Werner Syndrome Protein and DNA Replication
TLDR
Understanding WRN’s molecular role in timely and faithful DNA replication will further advance the understanding of the pathophysiology of WS.
Current advances in unraveling the function of the Werner syndrome protein.
The Werner syndrome gene: the molecular basis of RecQ helicase-deficiency diseases.
Enzymatic mechanism of the WRN helicase/nuclease.
The Werner syndrome protein is involved in RNA polymerase II transcription.
TLDR
A role for WRNp in RNA pol II transcription, possibly as a transcriptional activator, is assessed by analyzing the efficiency of basal transcription in WS lymphoblastoid cell lines that carry homozygous WRN mutations.
The Werner syndrome protein at the crossroads of DNA repair and apoptosis
Functional deficit associated with a missense Werner syndrome mutation.
The premature ageing syndrome protein, WRN, is a 3′→5′ exonuclease
TLDR
To test the prediction that WRN is an exonuclease, tagged recombinant wild-type and mutant WRN proteins were produced and statistical sequence analyses showed subtle but significant similarities between WRN and several 3′→5′ exonucleases.
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References

SHOWING 1-10 OF 26 REFERENCES
Positional Cloning of the Werner's Syndrome Gene
TLDR
The identification of a mutated putative helicase as the gene product of the WS gene suggests that defective DNA metabolism is involved in the complex process of aging in WS patients.
Mutations in the consensus helicase domains of the Werner syndrome gene. Werner's Syndrome Collaborative Group.
TLDR
Results confirm that mutations in the WRN gene are responsible for Werner syndrome and suggest that WS is the result of complete loss of function of theWRN gene product.
Mutator phenotype of Werner syndrome is characterized by extensive deletions.
TLDR
The biochemical and molecular characterization of spontaneous mutations at the X chromosome-linked hypoxanthine phosphoribosyltransferase (HPRT) locus in 6-thioguanine-resistant Werner syndrome cells suggest that an elevated somatic mutation rate, and particularly deletions, may play pathogenetically important roles in WS and in several associated age-dependent human disease processes.
Human xeroderma pigmentosum group D gene encodes a DMA helicase
XERODERMA pigmentosum (XP), a genetically heterogeneous human disease, results from a defect in nucleotide excision repair of ultraviolet-damaged DNA. XP patients are extremely sensitive to sunlight…
The Bloom's syndrome gene product is homologous to RecQ helicases
RAD26, the functional S. cerevisiae homolog of the Cockayne syndrome B gene ERCC6.
TLDR
The findings suggest that TCR in lower eukaryotes is not very important for cell survival and that the global genome repair pathway of NER is the major determinant of cellular resistance to genotoxicity.
Impaired S-phase transit of Werner syndrome cells expressed in lymphoblastoid cell lines.
Homozygous and compound heterozygous mutations at the Werner syndrome locus.
TLDR
Nine new WRN mutations in 10 additional WS patients, both Japanese and Caucasian, are described, which include three compound heterozygotes (one Japanese and two Caucasian).
Excess of rare cancers in Werner syndrome (adult progeria).
  • M. Goto, R. W. Miller, Y. Ishikawa, H. Sugano
  • Medicine
    Cancer epidemiology, biomarkers & prevention : a publication of the American Association for Cancer Research, cosponsored by the American Society of Preventive Oncology
  • 1996
TLDR
The high frequency of thyroid cancer and melanoma in Japanese may be related to a report of linkage disequilibrium with the WRN gene in Japanese but not in Caucasians and to haplotype differences within and between the two races, suggesting multiple independent mutations.
Cytogenetics of Werner's syndrome cultured skin fibroblasts: variegated translocation mosaicism.
TLDR
Skin fibroblast-like cells from patients with Werner's syndrome regularly demonstrate frequent pseudodiploidy involving variable structural rearrangements that are clonal: variegated translocation mosaicism (VTM), and the relationship between VTM and the reduced growth potential of Werner's Syndrome FL cells is not yet understood.
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