Human premature aging syndromes and genomic instability

Mechanisms of Ageing and Development

Volumn 123, Issue 8, 2002, Pages 987-993

  Bohr, Vilhelm A ^a  

^a NATIONAL INSTITUTE ON AGING   (United States)

Author keywords

DNA Repair; Genomic instability; Human premature aging diseases

Indexed keywords

PROTEIN; UNCLASSIFIED DRUG; WERNER PROTEIN;

CANCER; CARDIOVASCULAR DISEASE; CLINICAL FEATURE; COCKAYNE SYNDROME; DNA REPAIR; DNA TRANSCRIPTION; GENETIC STABILITY; GENOME; HUMAN; OSTEOPOROSIS; PREMATURE AGING; PRIORITY JOURNAL; PROGERIA; PROTEIN FUNCTION; PROTEIN PROTEIN INTERACTION; REVIEW; ROTHMUND THOMSON SYNDROME; VERNER MORRISON SYNDROME; XERODERMA PIGMENTOSUM;

EID: 0037198074     PISSN: 00476374     EISSN: None     Source Type: Journal    
DOI: 10.1016/S0047-6374(02)00039-8     Document Type: Article

References (46)

1. Reduced RNA polymerase II transcription in intact and permeabilized Cockayne syndrome group B cells
2. The Werner syndrome protein is involved in RNA polymerase II transcription
3. Genomic heterogeneity of nucleotide excision repair
4. DNA repair and mutagenesis in Werner syndrome
5. Functional and physical interaction between WRN helicase and human replication protein A
6. Werner syndrome protein interacts with human flap endonuclease 1 and stimulates its cleavage activity
7. p53 Modulates the exonuclease activity of Werner syndrome protein
8. Unwinding of a DNA triple helix by the Werner and Bloom syndrome helicases
9. The Werner's Syndrome Protein (WRN) translocates Holliday Junctions in vitro and co-localises with RPA upon replication arrest
10. Defective transcription-coupled repair of oxidative base damage in Cockayne syndrome patients from XP group G
11. Ku complex interacts with and stimulates the Werner protein
12. Repair pathways for processing of 8-oxoguanine in DNA by mammalian cell extracts
13. Repair of 8-oxoguanine in DNA is deficient in Cockayne syndrome group B cells
14. Cockayne syndrome - A primary defect in DNA repair, transcription, both or neither?
15. Mutator phenotype of Werner syndrome is characterized by extensive deletions
16. Two related superfamilies of putative helicases involved in replication, recombination, repair and expression of DNA and RNA genomes
17. The Werner syndrome protein is a DNA helicase
18. RecQ DNA helicase is a suppressor of illegitimate recombination in Escherichia coli
19. WRN or telomerase constructs reverse 4-nitroquinoline 1-oxide sensitivity in transformed Werner syndrome fibroblasts
20. The Saccharomyces cerevisiae WRN homolog Sgs1p participates in telomere maintenance in cells lacking telomerase
21. Functional interaction between the Werner Syndrome protein and DNA polymerase delta
22. Covalent modification of the Werner's syndrome gene product with the ubiquitin-related protein, SUMO-1
23. DNA damage and repair in telomeres: Relation to aging
24. The Werner syndrome gene product co-purifies with the DNA replication complex and interacts with PCNA and topoisomerase I
25. Functional interaction between Ku and the Werner syndrome protein in DNA end processing
26. The pathobiology of the Werner syndrome
27. Failure of RNA synthesis to recover after UV irradiation: And early defect in cells from individuals with Cockayne's syndrome and xeroderma pigmentosum
28. An apoptosis-inducing genotoxin differentiates heterozygotic carriers for Werner helicase mutations from wild-type and homozygous mutants
29. The human CSB (ERCC6) gene corrects the transcription-coupled repair defect in the CHO cell mutant UV61
30. SWI2/SNF2 and related proteins: ATP-driven motors that disrupt protein-DNA interactions?
31. Impaired S-phase transit of Werner syndrome cells expressed in lymphoblastoid cells
32. Werner syndrome cells are sensitive to DNA cross-linking drugs
33. Accelerated loss of telomeric repeats may not explain accelerated replicative decline of Werner syndrome cells
34. Human transcription-repair coupling factor CSB/ERCC6 is a DNA-stimulated ATPase but is not a helicase and does not disrupt the ternary transcription complex of stalled RNA polymerase II
35. RNA polymerase II stalled at a thymine dimer: Footprint and effect of excision repair
36. Characterization of Werner syndrome protein DNA helicase activity: Directionality, substrate dependence and stimulation by replication protein A
37. p53-mediated apoptosis is attenuated in Werner syndrome cells
38. Genetic instability as the primary cause of human aging
39. Deletional mutations are the basic cause of aging: Historical perspectives
40. DNA helicase activity in Werner's syndrome gene product synthesized in a baculovirus system
41. Werner protein recruits DNA polymerase delta to the nucleolus
42. Recruitment of the putative transcription-repair coupling factor CSB/ERCC6 to RNA polymerase II elongation complexes
43. ERCC6 a member of a subfamily of putative helicases is involved in Cockayne'ss syndrome and preferential repair of active genes
44. The Cockayne Syndrome group B gene product is involved in general genome base excision repair of 8-hydroxyguanine in DNA
45. The genetic defect in Cockayne syndrome is associated with a defect in repair of UV-induced DNA damage in transcriptionally active DNA
46. Telomerase prevents the accelerated cell ageing of Werner syndrome fibroblasts