Direct and indirect roles of RECQL4 in modulating base excision repair capacity (2009)
Schurman, Shepherd H., Hedayati, Mohammad, Wang, ZhengMing, Singh, Dharmendra K., Speina, Elzbieta, Zhang, Yongqing, ...
RECQL4 is a human RecQ helicase which is mutated in approximately two-thirds of individuals with Rothmund–Thomson syndrome (RTS), a disease characterized at the cellular level by chromosomal...
Base excision repair of oxidative DNA damage and association with cancer and aging (2009)
Maynard, Scott, Schurman, Shepherd H., Harboe, Charlotte, Bohr, Vilhelm A.
Aging has been associated with damage accumulation in the genome and with increased cancer incidence. Reactive oxygen species (ROS) are produced from endogenous sources, most notably the oxidative...
Hyun, Moonjung, Lee, Jihyun, Lee, Kyungjin, May, Alfred, Bohr, Vilhelm A., Ahn, Byungchan
DNA repair is an important mechanism by which cells maintain genomic integrity. Decline in DNA repair capacity or defects in repair factors are thought to contribute to premature aging in mammals....
Bukowy, Zuzanna, Harrigan, Jeanine A., Ramsden, Dale A., Tudek, Barbara, Bohr, Vilhelm A., Stevnsner, Tinna
Werner syndrome (WS) is a premature aging disorder caused by mutations in the WS gene (WRN). Although WRN has been suggested to play an important role in DNA metabolic pathways, such as...
Weissman, Lior, Jo, Dong-Gyu, Sørensen, Martin M., Markesbery, William R., Mattson, Mark P., ...
Oxidative stress is thought to play a role in the pathogenesis of Alzheimer's disease (AD) and increased oxidative DNA damage has been observed in brain tissue from AD patients. Base excision repair...
Cockayne syndrome protein B interacts with and is phosphorylated by c-Abl tyrosine kinase (2007)
Imam, Syed Z., Indig, Fred E., Cheng, Wen-Hsing, Saxena, Satya P., Stevnsner, Tinna, Kufe, Donald, ...
The Cockayne Syndrome group B (CSB) protein plays important roles in transcription, transcription-coupled nucleotide excision repair and base excision DNA repair. c-Abl kinase also plays a role in...
Wong, Heng-Kuan, Muftuoglu, Meltem, Beck, Gad, Imam, Syed Z., Bohr, Vilhelm A., Wilson, David M.
The Cockayne syndrome B (CSB) protein—defective in a majority of patients suffering from the rare autosomal disorder CS—is a member of the SWI2/SNF2 family with roles in DNA repair and...
Human premature aging, DNA repair and RecQ helicases (2007)
Brosh, Robert M., Bohr, Vilhelm A.
Genomic instability leads to mutations, cellular dysfunction and aberrant phenotypes at the tissue and organism levels. A number of mechanisms have evolved to cope with endogenous or exogenous stress...
Cockayne syndrome group B protein has novel strand annealing and exchange activities (2006)
Muftuoglu, Meltem, Sharma, Sudha, Thorslund, Tina, Stevnsner, Tinna, Soerensen, Martin M., Brosh, Robert M., ...
Cockayne syndrome (CS) is a rare inherited human genetic disorder characterized by UV sensitivity, severe neurological abnormalities and prageroid symptoms. The CS complementation group B (CSB)...
Cheng, Wen-Hsing, Kusumoto, Rika, Opresko, Patricia L., Sui, XiuFen, Huang, Shurong, Nicolette, Matthew L., ...
Cells deficient in the Werner syndrome protein (WRN) or BRCA1 are hypersensitive to DNA interstrand cross-links (ICLs), whose repair requires nucleotide excision repair (NER) and homologous...
Harrigan, Jeanine A., Wilson, David M., Prasad, Rajendra, Opresko, Patricia L., Beck, Gad, May, Alfred, ...
Genome instability is a characteristic of cancer and aging, and is a hallmark of the premature aging disorder Werner syndrome (WS). Evidence suggests that the Werner syndrome protein (WRN)...
Oxidative damage in telomeric DNA disrupts recognition by TRF1 and TRF2 (2005)
Opresko, Patricia L., Fan, Jinshui, Danzy, Shamika, Wilson, David M., Bohr, Vilhelm A.
The ends of linear chromosomes are capped by protein–DNA complexes termed telomeres. Telomere repeat binding factors 1 and 2 (TRF1 and TRF2) bind specifically to duplex telomeric DNA and are...
Phosphorylation of human oxoguanine DNA glycosylase ({alpha}-OGG1) modulates its function (2005)
Hu, Jingping, Imam, Syed Z., Hashiguchi, Kazunari, Bohr, Vilhelm A.
Oxoguanine DNA glycosylase (OGG1) initiates the repair of 8-oxoguanine (8-oxoG), a major oxidative DNA base modification that has been directly implicated in cancer and aging. OGG1 functions in the...
Von Kobbe, Cayetano, Harrigan, Jeanine A., Schreiber, Valérie, Stiegler, Patrick, Piotrowski, Jason, Dawut, Lale, ...
Werner syndrome (WS) is a genetic premature aging disorder in which patients appear much older than their chronological age. The gene mutated in WS encodes a nuclear protein (WRN) which possesses...
Werner syndrome and the function of the werner protein (2003)
Opresko, Patricia L., Cheng, Wen-Hsing, Von Kobbe, Cayetano, Harrigan, Jeanine A., Bohr, Vilhelm A.
Werner syndrome (WS) is a hallmark premature aging disease, in which the patients appear much older than their chronological age, and exhibit many of the clinical signs and symptoms of normal aging...
Opresko, Patricia L., Cheng, Wen-Hsing, Von Kobbe, Cayetano, Harrigan, Jeanine A., Bohr, Vilhelm A.
Werner syndrome (WS) is a hallmark premature aging disease, in which the patients appear much older than their chronological age, and exhibit many of the clinical signs and symptoms of normal aging...
Christiansen, Mette, Stevnsner, Tinna, Modin, Charlotte, Martensen, Pia M., Brosh, Robert M., Bohr, Vilhelm A.
The rare inherited human genetic disorder Cockayne syndrome (CS) is characterized by developmental abnormalities, UV sensitivity and premature aging. The cellular and molecular phenotypes of CS...
Werner syndrome and the function of the werner protein (2003)
Opresko, Patricia L., Cheng, Wen-Hsing, Von Kobbe, Cayetano, Harrigan, Jeanine A., Bohr, Vilhelm A.
Werner syndrome (WS) is a hallmark premature aging disease, in which the patients appear much older than their chronological age, and exhibit many of the clinical signs and symptoms of normal aging...
Karmakar, Parimal, Snowden, Carey M., Ramsden, Dale A., Bohr, Vilhelm A.
The human Werner syndrome protein, WRN, is a member of the RecQ helicase family and contains 3′→5′ helicase and 3′→5′ exonuclease activities. Recently, we showed that the exonuclease...
Selzer, Rebecca R., Nyaga, Simon, Tuo, Jingsheng, May, Alfred, Muftuoglu, Meltem, Christiansen, Mette, ...
Cockayne syndrome (CS) is a rare inherited human genetic disorder characterized by UV sensitivity, developmental abnormalities and premature aging. The cellular and molecular phenotypes of CS include...
A functional interaction of Ku with Werner exonuclease facilitates digestion of damaged DNA (2001)
Orren, David K., Machwe, Amrita, Karmakar, Parimal, Piotrowski, Jason, Cooper, Marcus P., Bohr, Vilhelm A.
Werner syndrome (WS) is a premature aging disorder where the affected individuals appear much older than their chronological age. The single gene that is defective in WS encodes a protein (WRN) that...
Søe, Kent, Dianov, Grigory, Nasheuer, Heinz-Peter, Bohr, Vilhelm A., Grosse, Frank, Stevnsner, Tinna
Several recent studies have shown that human topoisomerase I (htopoI) can recognize various DNA lesions and thereby form a covalent topoisomerase I–DNA complex, which is known to be detrimental to...
The Bloom's and Werner's syndrome proteins are DNA structure-specific helicases (2001)
Mohaghegh, Payam, Karow, Julia K., Brosh, Robert M., Bohr, Vilhelm A., Hickson, Ian D.
BLM and WRN, the products of the Bloom’s and Werner’s syndrome genes, are members of the RecQ family of DNA helicases. Although both have been shown previously to unwind simple, partial duplex...
Potent inhibition of Werner and Bloom helicases by DNA minor groove binding drugs (2000)
Brosh Jr, Robert M., Karow, Julia K., White, E. James, Shaw, Natalie D., Hickson, Ian D., Bohr, Vilhelm A.
Maintenance of genomic integrity is vital to all organisms. A number of human genetic disorders, including Werner Syndrome, Bloom Syndrome and Rothmund-Thomson Syndrome, exhibit genomic instability...
Ku complex interacts with and stimulates the Werner protein (2000)
Cooper, Marcus P., Machwe, Amrita, Orren, David K., Brosh, Robert M., Ramsden, Dale, Bohr, Vilhelm A.
Machwe, Amrita, Ganunis, Ruth, Bohr, Vilhelm A., Orren, David K.
Individuals with mutations in the WRN gene suffer from Werner syndrome, a disease with early onset of many characteristics of normal aging. The WRN protein (WRNp) functions in DNA metabolism, as the...
Potent inhibition of Werner and Bloom helicases by DNA minor groove binding drugs (2000)
Brosh, Robert M., Karow, Julia K., White, E. James, Shaw, Natalie D., Hickson, Ian D., Bohr, Vilhelm A.
Maintenance of genomic integrity is vital to all organisms. A number of human genetic disorders, including Werner Syndrome, Bloom Syndrome and Rothmund–Thomson Syndrome, exhibit genomic instability...
A new xeroderma pigmentosum group C poly(AT) insertion/deletion polymorphism (2000)
Khan, Sikandar G., Metter, E.Jeffrey, Tarone, Robert E., Bohr, Vilhelm A., Grossman, Lawrence, Hedayati, Mohammad, ...
We found a common biallelic polymorphism (PAT) in the xeroderma pigmentosum complementation group C (XPC) DNA repair gene consisting of an insertion of 83 bases of A and T [poly(AT)] and a...
Transcription-coupled repair is inducible in hamster cells (2000)
Germanier, Maryse, Defais, Martine, Bohr, Vilhelm A., Larminat, Florence
In mammalian cells, the rate of nucleotide excision repair of UV dimers is heterogeneous throughout the genome, with repair occurring more rapidly in the transcribed strand of active genes than in...
Sunesen, Morten, Selzer, Rebecca R., Brosh Jr, Robert M., Balajee, Adayabalam S., Stevnsner, Tinna, Bohr, Vilhelm A.
Cockayne syndrome (CS) is a human genetic disorder characterized by post-natal growth failure, neurological abnormalities and premature aging. CS cells exhibit high sensitivity to UV light, delayed...
DNA repair in a UV resistant Chinese hamster ovary cell line (1995)
Petersen, Lone Nørgård, Stevnsner, Tinna, Bohr, Vilhelm A.
We have established an ultraviolet (UV) resistant Chinese hamster ovary (CHO) cell line B11UVres by repetitive UV exposure of the CHO cell line B11. We have characterized the resistant cell line with...
Link, Charles J., Evans, Michele K., Cook, John A., Muldoon, Rebecca, Stevnsner, Tinna, Bohr, Vilhelm A.
We have studied the effect of caffeine on gene- and strandspecific DNA repair after exposure of Chinese hamster ovary cells and human xeroderma pigmentosum complementation group C (XPC) cells to...
Wassermann, Karsten, O'Connor, Patrick M., Jackman, Joany, May, Alfred, Bohr, Vilhelm A.
Recently, it has been demonstrated that N-alkylpurines produced by nitrogen mustard are excised more rapidly from actively transcribing genes compared to non-coding regions of the overall genome....
Stevnsner, Tinna, Ding, Ruchuang, Smulson, Mark, Bohr, Vilhelm A.
The role of the enzyme poly(adenosine diphosphateribose) polymerase (PADPRP) in DNA repair at the level of the gene was investigated with human HeLa cells in which PADPRP antisense transcripts are...
Role of the human ERCC-1 gene in gene-specific repair of cisplatin-induced DNA damage (1994)
Larminat, Florence, Bohr, Vilhelm A.
The human excision repair gene ERCC-1 gene restores normal resistance to UV and mitomycin C in excision repair deficient Chinese hamster ovary cells of complementation group 1. To investigate the...
Zhen, Weiping, Evans, Michele K., Haggerty, Cynthia M., Bohr, Vilhelm A.
Cisplatin is a chemotherapeutic agent known to cause DNA damage. The cytotoxicity of this drug is believed to result from the formation of DNA intrastrand adducts (IA) and DNA interstrand crosslinks...
Pirsel, Miroslav, Bohr, Vilhelm A.
Chinese hamster ovary CHO-B11 cells were exposed to methyl methanesulfonate (MMS) and the formation and repair of N-methylpurines were measured in the endogenous dihydrofolate reductase (DHFR) gene...
Stevnsner, Tinna, May, Alfred, Petersen, Lone Nørgård, Larminat, Florence, Pirsel, Miroslav, Bohr, Vilhelm. A.
We have measured the DNA damage formation and repair in the ribosomal and the dihydrofolate reductase (DHFR) genes after treatment of hamster cells with different types of DNA damaging agents. In...
Studies on the role of topoisomerases in general, gene- and strand-specific DNA repair (1993)
Stevnsner, Tinna, Bohr, Vilhelm A.
Using specific inhibitors we have assessed the role of topoisomerases I and II in DNA repair of the overall genome and in both strands of an essential gene, the dihydrofolate reductase (DHFR) gene in...
Repair of mitochondrial DNA after various types of DNA damage in Chinese hamster ovary cells (1992)
LeDoux, Susan P., Wilson, Glenn L., Beecham, Edward J., Stevnsner, Tinna, Wassermann, Karsten, Bohr, Vilhelm A.
Using methodology recently developed to assess gene-specific DNA repair, we have demonstrated that it is possible not only to study mitochondrial DNA repair, but also directly to compare...
Link, Charles J., Mitchell, David L., Nairn, Rodney S., Bohr, Vilhelm A.
A non-enzymatic method that was previously shown to create single-strand DNA breaks at the location of (6–4) photo-products in the overall genome was adapted to measure (6–4) photoproducts at the...
ERCC1 and ERCC2 Expression in Malignant Tissues From Ovarian Cancer Patients (1992)
Dabholkar, Meenakshi, Bostick-Bruton, Frieda, Weber, Christine, Bohr, Vilhelm A., Egwuagu, Charles, Reed, Eddie
Background: ERCC1 and ERCC2 are human DNA repair genes that are associated with in vitro resistance to selected DNA-damaging agents. Purpose: Fresh tumor tissues from 26 patients with ovarian cancer...
Bohr, Vilhelm A., Chu, Ernest H.Y., Van Duin, Marcel, Hanawalt, Philip C., Okumoto, Diane S.
The pattern of preferential DNA repair of UV-induced pyr1n1d1ne dimers was studied in repair-deficient Chinese hamster ovary (CHO) cells transfected with the human excision repair gene, ERCC-1....
Preferential DNA repair in active genes / (1987)
Thesis (doctoral)--Københavns universitet, 1987.
DNA repair in the metallothionein gene increases with transcriptional activation (1987)
Okumoto, Diane S., Bohr, Vilhelm A.
We have studied DNA repair in the Chinese Hamster Ovary (CHO) metallothionein (MT) gene after UV-light induced damage. The repair was examined comparatively with or without transcriptional activation...
Bohr, Vilhelm A., Hanawalt, Philip C.
We have previously demonstrated that the active dihydrofolate reductase (DHFR) gene is efficiently repaired in Chinese hamster ovary (CHO) cells which remove only a small fraction of u.v.-induced...
Novobiocin does not inhibit DNA repair in an activve gene (1986)
Bohr, Vilhelm A., Hanawalt, Philip C.
Novobiocin, an inhibitor of type II topoisomerase, has been reported to inhibit u.v.-induced DNA repair in a number of established mammalian cell lines; we have confirmed this general observation in...
Reduced RNA polymerase II transcription in intact and permeabilized Cockayne syndrome group B cells
Balajee, Adayabalam S., May, Alfred, Dianov, Grigory L., Friedberg, Errol C., Bohr, Vilhelm A.
Cockayne syndrome (CS) is characterized by increased photosensitivity, growth retardation, and neurological and skeletal abnormalities. The recovery of RNA synthesis is abnormally delayed in CS cells...
Winter, David B., Phung, Quy H., Umar, Asad, Baker, Sean M., Tarone, Robert E., Tanaka, Kiyoji, ...
Mutations are introduced into rearranged Ig variable genes at a frequency of 10−2 mutations per base pair by an unknown mechanism. Assuming that DNA repair pathways generate or remove mutations,...
The C-terminal Domain of p21 Inhibits Nucleotide Excision Repair In Vitro and In Vivo
Cooper, Marcus P., Balajee, Adayabalam S., Bohr, Vilhelm A.
The protein p21Cip1, Waf1, Sdi1 is a potent inhibitor of cyclin-dependent kinases (CDKs). p21 can also block DNA replication through its interaction with the proliferating cell nuclear antigen...
The Werner Syndrome Protein Is Involved in RNA Polymerase II Transcription
Balajee, Adayabalam S., Machwe, Amrita, May, Alfred, Gray, Matthew D., Oshima, Junko, Martin, George M., ...
Werner syndrome (WS) is a human progeroid syndrome characterized by the early onset of a large number of clinical features associated with the normal aging process. The complex molecular and cellular...
Brosh, Robert M., Balajee, Adayabalam S., Selzer, Rebecca R., Sunesen, Morten, De Santis, Luca Proietti, Bohr, Vilhelm A.
Cockayne syndrome (CS) is a human genetic disorder characterized by UV sensitivity, developmental abnormalities, and premature aging. Two of the genes involved, CSA and CSB, are required for...
A functional interaction of Ku with Werner exonuclease facilitates digestion of damaged DNA
Orren, David K., Machwe, Amrita, Karmakar, Parimal, Piotrowski, Jason, Cooper, Marcus P., Bohr, Vilhelm A.
Werner syndrome (WS) is a premature aging disorder where the affected individuals appear much older than their chronological age. The single gene that is defective in WS encodes a protein (WRN) that...
The Bloom’s and Werner’s syndrome proteins are DNA structure-specific helicases
Mohaghegh, Payam, Karow, Julia K., Brosh Jr, Robert M., Bohr, Vilhelm A., Hickson, Ian D.
BLM and WRN, the products of the Bloom’s and Werner’s syndrome genes, are members of the RecQ family of DNA helicases. Although both have been shown previously to unwind simple, partial duplex...
Søe, Kent, Dianov, Grigory, Nasheuer, Heinz-Peter, Bohr, Vilhelm A., Grosse, Frank, Stevnsner, Tinna
Several recent studies have shown that human topoisomerase I (htopoI) can recognize various DNA lesions and thereby form a covalent topoisomerase I–DNA complex, which is known to be detrimental to...
Selzer, Rebecca R., Nyaga, Simon, Tuo, Jingsheng, May, Alfred, Muftuoglu, Meltem, Christiansen, Mette, ...
Cockayne syndrome (CS) is a rare inherited human genetic disorder characterized by UV sensitivity, developmental abnormalities and premature aging. The cellular and molecular phenotypes of CS include...
Machwe, Amrita, Ganunis, Ruth, Bohr, Vilhelm A., Orren, David K.
Individuals with mutations in the WRN gene suffer from Werner syndrome, a disease with early onset of many characteristics of normal aging. The WRN protein (WRNp) functions in DNA metabolism, as the...
Potent inhibition of Werner and Bloom helicases by DNA minor groove binding drugs
Brosh Jr, Robert M., Karow, Julia K., White, E. James, Shaw, Natalie D., Hickson, Ian D., Bohr, Vilhelm A.
Maintenance of genomic integrity is vital to all organisms. A number of human genetic disorders, including Werner Syndrome, Bloom Syndrome and Rothmund–Thomson Syndrome, exhibit genomic instability...
Molecular characterization of an acidic region deletion mutant of Cockayne syndrome group B protein
Sunesen, Morten, Selzer, Rebecca R., Brosh, Robert M., Balajee, Adayabalam S., Stevnsner, Tinna, Bohr, Vilhelm A.
Cockayne syndrome (CS) is a human genetic disorder characterized by post-natal growth failure, neurological abnormalities and premature aging. CS cells exhibit high sensitivity to UV light, delayed...
Transcription-coupled repair is inducible in hamster cells
Germanier, Maryse, Defais, Martine, Bohr, Vilhelm A., Larminat, Florence
In mammalian cells, the rate of nucleotide excision repair of UV dimers is heterogeneous throughout the genome, with repair occurring more rapidly in the transcribed strand of active genes than in...
Brosh, Robert M., Von Kobbe, Cayetano, Sommers, Joshua A., Karmakar, Parimal, Opresko, Patricia L., Piotrowski, Jason, ...
Werner syndrome (WS) is a human premature aging disorder characterized by chromosomal instability. The cellular defects of WS presumably reflect compromised or aberrant function of a DNA metabolic...
Karmakar, Parimal, Snowden, Carey M., Ramsden, Dale A., Bohr, Vilhelm A.
The human Werner syndrome protein, WRN, is a member of the RecQ helicase family and contains 3′→5′ helicase and 3′→5′ exonuclease activities. Recently, we showed that the exonuclease...
Human DNA polymerase β initiates DNA synthesis during long-patch repair of reduced AP sites in DNA
Podlutsky, Andrej Ja., Dianova, Irina I., Podust, Vladimir N., Bohr, Vilhelm A., Dianov, Grigory L.
Simple base damages are repaired through a short-patch base excision pathway where a single damaged nucleotide is removed and replaced. DNA polymerase β (Pol β) is responsible for the repair...
Christiansen, Mette, Stevnsner, Tinna, Modin, Charlotte, Martensen, Pia M., Brosh, Robert M., Bohr, Vilhelm A.
The rare inherited human genetic disorder Cockayne syndrome (CS) is characterized by developmental abnormalities, UV sensitivity and premature aging. The cellular and molecular phenotypes of CS...
Cheng, Wen-Hsing, Kobbe, Cayetano Von, Opresko, Patricia L., Fields, Kesha M., Ren, Jian, Kufe, Donald, ...
The Werner syndrome protein (WRN) is a caretaker of the human genome, and the Abl kinase is a regulator of the DNA damage response. Aberrant DNA repair has been linked to the development of cancer....
Gene expression profiling in Werner syndrome closely resembles that of normal aging
Kyng, Kasper J., May, Alfred, Kølvraa, Steen, Bohr, Vilhelm A.
Werner syndrome (WS) is a premature aging disorder, displaying defects in DNA replication, recombination, repair, and transcription. It has been hypothesized that several WS phenotypes are secondary...
Von Kobbe, Cayetano, Harrigan, Jeanine A., May, Alfred, Opresko, Patricia L., Dawut, Lale, Cheng, Wen-Hsing, ...
A defect in the Werner syndrome protein (WRN) leads to the premature aging disease Werner syndrome (WS). Hallmark features of cells derived from WS patients include genomic instability and...
Ku complex interacts with and stimulates the Werner protein
Cooper, Marcus P., Machwe, Amrita, Orren, David K., Brosh, Robert M., Ramsden, Dale, Bohr, Vilhelm A.
Werner syndrome (WS) is the hallmark premature aging disorder in which affected humans appear older than their chronological age. The protein WRNp, defective in WS, has helicase function,...
Von Kobbe, Cayetano, Harrigan, Jeanine A., Schreiber, Valérie, Stiegler, Patrick, Piotrowski, Jason, Dawut, Lale, ...
Werner syndrome (WS) is a genetic premature aging disorder in which patients appear much older than their chronological age. The gene mutated in WS encodes a nuclear protein (WRN) which possesses...
Oxidative damage in telomeric DNA disrupts recognition by TRF1 and TRF2
Opresko, Patricia L., Fan, Jinshui, Danzy, Shamika, Wilson, David M., Bohr, Vilhelm A.
The ends of linear chromosomes are capped by protein–DNA complexes termed telomeres. Telomere repeat binding factors 1 and 2 (TRF1 and TRF2) bind specifically to duplex telomeric DNA and are...
Constantinou, Angelos, Tarsounas, Madalena, Karow, Julia K., Brosh, Robert M., Bohr, Vilhelm A., Hickson, Ian D., ...
Individuals affected by the autosomal recessive disorder Werner’s syndrome (WS) develop many of the symptoms characteristic of premature ageing. Primary fibroblasts cultured from WS patients...
Phosphorylation of human oxoguanine DNA glycosylase (α-OGG1) modulates its function
Hu, Jingping, Imam, Syed Z., Hashiguchi, Kazunari, Bohr, Vilhelm A.
Oxoguanine DNA glycosylase (OGG1) initiates the repair of 8-oxoguanine (8-oxoG), a major oxidative DNA base modification that has been directly implicated in cancer and aging. OGG1 functions in the...
The HRDC domain of BLM is required for the dissolution of double Holliday junctions
Wu, Leonard, Lung Chan, Kok, Ralf, Christine, Bernstein, Douglas A, Garcia, Patrick L, Bohr, Vilhelm A, ...
Bloom's syndrome is a hereditary cancer-predisposition disorder resulting from mutations in the BLM gene. In humans, BLM encodes one of five members of the RecQ helicase family. One function of BLM...
Thorslund, Tina, Von Kobbe, Cayetano, Harrigan, Jeanine A., Indig, Fred E., Christiansen, Mette, Stevnsner, Tinna, ...
Cockayne syndrome (CS) is a rare genetic disorder characterized as a segmental premature-aging syndrome. The CS group B (CSB) protein has previously been implicated in transcription-coupled repair,...
Cockayne syndrome group B protein has novel strand annealing and exchange activities
Muftuoglu, Meltem, Sharma, Sudha, Thorslund, Tina, Stevnsner, Tinna, Soerensen, Martin M., Brosh, Robert M., ...
Cockayne syndrome (CS) is a rare inherited human genetic disorder characterized by UV sensitivity, severe neurological abnormalities and prageroid symptoms. The CS complementation group B (CSB)...
The Werner syndrome protein operates in base excision repair and cooperates with DNA polymerase β
Harrigan, Jeanine A., Wilson, David M., Prasad, Rajendra, Opresko, Patricia L., Beck, Gad, May, Alfred, ...
Genome instability is a characteristic of cancer and aging, and is a hallmark of the premature aging disorder Werner syndrome (WS). Evidence suggests that the Werner syndrome protein (WRN)...
Cheng, Wen-Hsing, Kusumoto, Rika, Opresko, Patricia L., Sui, XiuFen, Huang, Shurong, Nicolette, Matthew L., ...
Cells deficient in the Werner syndrome protein (WRN) or BRCA1 are hypersensitive to DNA interstrand cross-links (ICLs), whose repair requires nucleotide excision repair (NER) and homologous...
Epigenetic inactivation of the premature aging Werner syndrome gene in human cancer
Agrelo, Ruben, Cheng, Wen-Hsing, Setien, Fernando, Ropero, Santiago, Espada, Jesus, Fraga, Mario F., ...
Werner syndrome (WS) is an inherited disorder characterized by premature onset of aging, genomic instability, and increased cancer incidence. The disease is caused by loss of function mutations of...
Reduced RNA polymerase II transcription in intact and permeabilized Cockayne syndrome group B cells
Balajee, Adayabalam S., May, Alfred, Dianov, Grigory L., Friedberg, Errol C., Bohr, Vilhelm A.
Cockayne syndrome (CS) is characterized by increased photosensitivity, growth retardation, and neurological and skeletal abnormalities. The recovery of RNA synthesis is abnormally delayed in CS cells...
Winter, David B., Phung, Quy H., Umar, Asad, Baker, Sean M., Tarone, Robert E., Tanaka, Kiyoji, ...
Mutations are introduced into rearranged Ig variable genes at a frequency of 10−2 mutations per base pair by an unknown mechanism. Assuming that DNA repair pathways generate or remove mutations,...
The C-terminal Domain of p21 Inhibits Nucleotide Excision Repair In Vitro and In Vivo
Cooper, Marcus P., Balajee, Adayabalam S., Bohr, Vilhelm A.
The protein p21Cip1, Waf1, Sdi1 is a potent inhibitor of cyclin-dependent kinases (CDKs). p21 can also block DNA replication through its interaction with the proliferating cell nuclear antigen...
The Werner Syndrome Protein Is Involved in RNA Polymerase II Transcription
Balajee, Adayabalam S., Machwe, Amrita, May, Alfred, Gray, Matthew D., Oshima, Junko, Martin, George M., ...
Werner syndrome (WS) is a human progeroid syndrome characterized by the early onset of a large number of clinical features associated with the normal aging process. The complex molecular and cellular...
Brosh, Robert M., Balajee, Adayabalam S., Selzer, Rebecca R., Sunesen, Morten, De Santis, Luca Proietti, Bohr, Vilhelm A.
Cockayne syndrome (CS) is a human genetic disorder characterized by UV sensitivity, developmental abnormalities, and premature aging. Two of the genes involved, CSA and CSB, are required for...
A functional interaction of Ku with Werner exonuclease facilitates digestion of damaged DNA
Orren, David K., Machwe, Amrita, Karmakar, Parimal, Piotrowski, Jason, Cooper, Marcus P., Bohr, Vilhelm A.
Werner syndrome (WS) is a premature aging disorder where the affected individuals appear much older than their chronological age. The single gene that is defective in WS encodes a protein (WRN) that...
The Bloom’s and Werner’s syndrome proteins are DNA structure-specific helicases
Mohaghegh, Payam, Karow, Julia K., Brosh Jr, Robert M., Bohr, Vilhelm A., Hickson, Ian D.
BLM and WRN, the products of the Bloom’s and Werner’s syndrome genes, are members of the RecQ family of DNA helicases. Although both have been shown previously to unwind simple, partial duplex...
Søe, Kent, Dianov, Grigory, Nasheuer, Heinz-Peter, Bohr, Vilhelm A., Grosse, Frank, Stevnsner, Tinna
Several recent studies have shown that human topoisomerase I (htopoI) can recognize various DNA lesions and thereby form a covalent topoisomerase I–DNA complex, which is known to be detrimental to...
Selzer, Rebecca R., Nyaga, Simon, Tuo, Jingsheng, May, Alfred, Muftuoglu, Meltem, Christiansen, Mette, ...
Cockayne syndrome (CS) is a rare inherited human genetic disorder characterized by UV sensitivity, developmental abnormalities and premature aging. The cellular and molecular phenotypes of CS include...
Machwe, Amrita, Ganunis, Ruth, Bohr, Vilhelm A., Orren, David K.
Individuals with mutations in the WRN gene suffer from Werner syndrome, a disease with early onset of many characteristics of normal aging. The WRN protein (WRNp) functions in DNA metabolism, as the...
Potent inhibition of Werner and Bloom helicases by DNA minor groove binding drugs
Brosh Jr, Robert M., Karow, Julia K., White, E. James, Shaw, Natalie D., Hickson, Ian D., Bohr, Vilhelm A.
Maintenance of genomic integrity is vital to all organisms. A number of human genetic disorders, including Werner Syndrome, Bloom Syndrome and Rothmund–Thomson Syndrome, exhibit genomic instability...
Molecular characterization of an acidic region deletion mutant of Cockayne syndrome group B protein
Sunesen, Morten, Selzer, Rebecca R., Brosh, Robert M., Balajee, Adayabalam S., Stevnsner, Tinna, Bohr, Vilhelm A.
Cockayne syndrome (CS) is a human genetic disorder characterized by post-natal growth failure, neurological abnormalities and premature aging. CS cells exhibit high sensitivity to UV light, delayed...
Transcription-coupled repair is inducible in hamster cells
Germanier, Maryse, Defais, Martine, Bohr, Vilhelm A., Larminat, Florence
In mammalian cells, the rate of nucleotide excision repair of UV dimers is heterogeneous throughout the genome, with repair occurring more rapidly in the transcribed strand of active genes than in...
Brosh, Robert M., Von Kobbe, Cayetano, Sommers, Joshua A., Karmakar, Parimal, Opresko, Patricia L., Piotrowski, Jason, ...
Werner syndrome (WS) is a human premature aging disorder characterized by chromosomal instability. The cellular defects of WS presumably reflect compromised or aberrant function of a DNA metabolic...
Karmakar, Parimal, Snowden, Carey M., Ramsden, Dale A., Bohr, Vilhelm A.
The human Werner syndrome protein, WRN, is a member of the RecQ helicase family and contains 3′→5′ helicase and 3′→5′ exonuclease activities. Recently, we showed that the exonuclease...
Human DNA polymerase β initiates DNA synthesis during long-patch repair of reduced AP sites in DNA
Podlutsky, Andrej Ja., Dianova, Irina I., Podust, Vladimir N., Bohr, Vilhelm A., Dianov, Grigory L.
Simple base damages are repaired through a short-patch base excision pathway where a single damaged nucleotide is removed and replaced. DNA polymerase β (Pol β) is responsible for the repair...
Christiansen, Mette, Stevnsner, Tinna, Modin, Charlotte, Martensen, Pia M., Brosh, Robert M., Bohr, Vilhelm A.
The rare inherited human genetic disorder Cockayne syndrome (CS) is characterized by developmental abnormalities, UV sensitivity and premature aging. The cellular and molecular phenotypes of CS...
Cheng, Wen-Hsing, Kobbe, Cayetano Von, Opresko, Patricia L., Fields, Kesha M., Ren, Jian, Kufe, Donald, ...
The Werner syndrome protein (WRN) is a caretaker of the human genome, and the Abl kinase is a regulator of the DNA damage response. Aberrant DNA repair has been linked to the development of cancer....
Gene expression profiling in Werner syndrome closely resembles that of normal aging
Kyng, Kasper J., May, Alfred, Kølvraa, Steen, Bohr, Vilhelm A.
Werner syndrome (WS) is a premature aging disorder, displaying defects in DNA replication, recombination, repair, and transcription. It has been hypothesized that several WS phenotypes are secondary...
Von Kobbe, Cayetano, Harrigan, Jeanine A., May, Alfred, Opresko, Patricia L., Dawut, Lale, Cheng, Wen-Hsing, ...
A defect in the Werner syndrome protein (WRN) leads to the premature aging disease Werner syndrome (WS). Hallmark features of cells derived from WS patients include genomic instability and...
Ku complex interacts with and stimulates the Werner protein
Cooper, Marcus P., Machwe, Amrita, Orren, David K., Brosh, Robert M., Ramsden, Dale, Bohr, Vilhelm A.
Werner syndrome (WS) is the hallmark premature aging disorder in which affected humans appear older than their chronological age. The protein WRNp, defective in WS, has helicase function,...
Von Kobbe, Cayetano, Harrigan, Jeanine A., Schreiber, Valérie, Stiegler, Patrick, Piotrowski, Jason, Dawut, Lale, ...
Werner syndrome (WS) is a genetic premature aging disorder in which patients appear much older than their chronological age. The gene mutated in WS encodes a nuclear protein (WRN) which possesses...
Oxidative damage in telomeric DNA disrupts recognition by TRF1 and TRF2
Opresko, Patricia L., Fan, Jinshui, Danzy, Shamika, Wilson, David M., Bohr, Vilhelm A.
The ends of linear chromosomes are capped by protein–DNA complexes termed telomeres. Telomere repeat binding factors 1 and 2 (TRF1 and TRF2) bind specifically to duplex telomeric DNA and are...
Constantinou, Angelos, Tarsounas, Madalena, Karow, Julia K., Brosh, Robert M., Bohr, Vilhelm A., Hickson, Ian D., ...
Individuals affected by the autosomal recessive disorder Werner’s syndrome (WS) develop many of the symptoms characteristic of premature ageing. Primary fibroblasts cultured from WS patients...
Phosphorylation of human oxoguanine DNA glycosylase (α-OGG1) modulates its function
Hu, Jingping, Imam, Syed Z., Hashiguchi, Kazunari, Bohr, Vilhelm A.
Oxoguanine DNA glycosylase (OGG1) initiates the repair of 8-oxoguanine (8-oxoG), a major oxidative DNA base modification that has been directly implicated in cancer and aging. OGG1 functions in the...
The HRDC domain of BLM is required for the dissolution of double Holliday junctions
Wu, Leonard, Lung Chan, Kok, Ralf, Christine, Bernstein, Douglas A, Garcia, Patrick L, Bohr, Vilhelm A, ...
Bloom's syndrome is a hereditary cancer-predisposition disorder resulting from mutations in the BLM gene. In humans, BLM encodes one of five members of the RecQ helicase family. One function of BLM...
Cockayne Syndrome Group B Cellular and Biochemical Functions
Licht, Cecilie Löe, Stevnsner, Tinna, Bohr, Vilhelm A.
The devastating genetic disorder Cockayne syndrome (CS) arises from mutations in the CSA and CSB genes. CS is characterized by progressive multisystem degeneration and is classified as a segmental...
Thorslund, Tina, Von Kobbe, Cayetano, Harrigan, Jeanine A., Indig, Fred E., Christiansen, Mette, Stevnsner, Tinna, ...
Cockayne syndrome (CS) is a rare genetic disorder characterized as a segmental premature-aging syndrome. The CS group B (CSB) protein has previously been implicated in transcription-coupled repair,...
Cockayne syndrome group B protein has novel strand annealing and exchange activities
Muftuoglu, Meltem, Sharma, Sudha, Thorslund, Tina, Stevnsner, Tinna, Soerensen, Martin M., Brosh, Robert M., ...
Cockayne syndrome (CS) is a rare inherited human genetic disorder characterized by UV sensitivity, severe neurological abnormalities and prageroid symptoms. The CS complementation group B (CSB)...
The Werner syndrome protein operates in base excision repair and cooperates with DNA polymerase β
Harrigan, Jeanine A., Wilson, David M., Prasad, Rajendra, Opresko, Patricia L., Beck, Gad, May, Alfred, ...
Genome instability is a characteristic of cancer and aging, and is a hallmark of the premature aging disorder Werner syndrome (WS). Evidence suggests that the Werner syndrome protein (WRN)...
Cheng, Wen-Hsing, Kusumoto, Rika, Opresko, Patricia L., Sui, XiuFen, Huang, Shurong, Nicolette, Matthew L., ...
Cells deficient in the Werner syndrome protein (WRN) or BRCA1 are hypersensitive to DNA interstrand cross-links (ICLs), whose repair requires nucleotide excision repair (NER) and homologous...
Epigenetic inactivation of the premature aging Werner syndrome gene in human cancer
Agrelo, Ruben, Cheng, Wen-Hsing, Setien, Fernando, Ropero, Santiago, Espada, Jesus, Fraga, Mario F., ...
Werner syndrome (WS) is an inherited disorder characterized by premature onset of aging, genomic instability, and increased cancer incidence. The disease is caused by loss of function mutations of...
Rosner, Karli, Winter, David B, Tarone, Robert E, Skovgaard, Gunhild Lange, Bohr, Vilhelm A, Gearhart, Patricia J
The third complementarity-determining region (CDR3) of immunoglobulin variable genes for the heavy chain (VH) has been shown to be shorter in length in hypermutated antibodies than in...
A role for WRN in telomere-based DNA damage responses
Eller, Mark S., Liao, Xiaodong, Liu, SuiYang, Hanna, Kendra, Bäckvall, Helena, Opresko, Patricia L., ...
Telomeres cap the ends of eukaryotic chromosomes and prevent them from being recognized as DNA breaks. We have shown that certain DNA damage responses induced during senescence and, at times of...
Mitochondrial Toxin 3-Nitropropionic Acid Induces Cardiac and Neurotoxicity Differentially in Mice
Gabrielson, Kathleen L., Hogue, Barbara A., Bohr, Vilhelm A., Cardounel, A. J., Nakajima, Waco, Kofler, Julia, ...
We investigated the effects of 3-nitropropionic acid (3NPA), a previously characterized neurotoxin, in four strains of mice to better understand the molecular basis of variable host responses to this...
Wong, Heng-Kuan, Muftuoglu, Meltem, Beck, Gad, Imam, Syed Z., Bohr, Vilhelm A., Wilson, David M.
The Cockayne syndrome B (CSB) protein—defective in a majority of patients suffering from the rare autosomal disorder CS—is a member of the SWI2/SNF2 family with roles in DNA repair and...
Cockayne syndrome protein B interacts with and is phosphorylated by c-Abl tyrosine kinase
Imam, Syed Z., Indig, Fred E., Cheng, Wen-Hsing, Saxena, Satya P., Stevnsner, Tinna, Kufe, Donald, ...
The Cockayne Syndrome group B (CSB) protein plays important roles in transcription, transcription-coupled nucleotide excision repair and base excision DNA repair. c-Abl kinase also plays a role in...
Weissman, Lior, Jo, Dong-Gyu, Sørensen, Martin M., Markesbery, William R., Mattson, Mark P., ...
Oxidative stress is thought to play a role in the pathogenesis of Alzheimer's disease (AD) and increased oxidative DNA damage has been observed in brain tissue from AD patients. Base excision repair...
Human premature aging, DNA repair and RecQ helicases
Brosh, Robert M., Bohr, Vilhelm A.
Genomic instability leads to mutations, cellular dysfunction and aberrant phenotypes at the tissue and organism levels. A number of mechanisms have evolved to cope with endogenous or exogenous stress...
Acetylation Regulates WRN Catalytic Activities and Affects Base Excision DNA Repair
Muftuoglu, Meltem, Kusumoto, Rika, Speina, Elzbieta, Beck, Gad, Cheng, Wen-Hsing, Bohr, Vilhelm A.
Longevity and resistance to stress correlate with DNA repair capacity in Caenorhabditis elegans
Hyun, Moonjung, Lee, Jihyun, Lee, Kyungjin, May, Alfred, Bohr, Vilhelm A., Ahn, Byungchan
DNA repair is an important mechanism by which cells maintain genomic integrity. Decline in DNA repair capacity or defects in repair factors are thought to contribute to premature aging in mammals....
Phung, Quy H., Winter, David B., Cranston, Aaron, Tarone, Robert E., Bohr, Vilhelm A., Fishel, Richard, ...
Rearranged immunoglobulin variable genes are extensively mutated after stimulation of B lymphocytes by antigen. Mutations are likely generated by an error-prone DNA polymerase, and the mismatch...
Bukowy, Zuzanna, Harrigan, Jeanine A., Ramsden, Dale A., Tudek, Barbara, Bohr, Vilhelm A., Stevnsner, Tinna
Werner syndrome (WS) is a premature aging disorder caused by mutations in the WS gene (WRN). Although WRN has been suggested to play an important role in DNA metabolic pathways, such as...
Cheng, Wen-Hsing, Muftic, Diana, Muftuoglu, Meltem, Dawut, Lale, Morris, Christa, Helleday, Thomas, ...
Werner syndrome (WS) is a human genetic disorder characterized by extensive clinical features of premature aging. Ataxia-telengiectasia (A-T) is a multisystem human genomic instability syndrome that...
Liu, Pingfang, Qian, Limin, Sung, Jung-Suk, Zheng, Li, Bogenhagen, Daniel F., ...
Repair of oxidative DNA damage in mitochondria was thought limited to short-patch base excision repair (SP-BER) replacing a single nucleotide. However, certain oxidative lesions cannot be processed...
Ahn, Byungchan, Lee, Jae Wan, Jung, Hana, Beck, Gad, Bohr, Vilhelm A.
WRN belongs to the RecQ family of DNA helicases and it plays a role in recombination, replication, telomere maintenance and long-patch base excision repair. Here, we demonstrate that WRN efficiently...