BLM

Summary

Gene Symbol: BLM
Description: Bloom syndrome RecQ like helicase
Alias: RECQ2, RECQL2, RECQL3, Bloom syndrome protein, Bloom syndrome, RecQ helicase-like, DNA helicase, RecQ-like type 2, recQ protein-like 3
Species: human
Products:     BLM

Top Publications

  1. Karmakar P, Seki M, Kanamori M, Hashiguchi K, Ohtsuki M, Murata E, et al. BLM is an early responder to DNA double-strand breaks. Biochem Biophys Res Commun. 2006;348:62-9 pubmed
    ..The defective protein in BS, BLM, is a member of the RecQ helicase family and is believed to function in various DNA transactions, including in ..
  2. Yankiwski V, Marciniak R, Guarente L, Neff N. Nuclear structure in normal and Bloom syndrome cells. Proc Natl Acad Sci U S A. 2000;97:5214-9 pubmed
    ..b>BLM, the protein altered in BS, is a RecQ DNA helicase...
  3. Guo R, Rigolet P, Ren H, Zhang B, Zhang X, Dou S, et al. Structural and functional analyses of disease-causing missense mutations in Bloom syndrome protein. Nucleic Acids Res. 2007;35:6297-310 pubmed
    ..Missense mutations have been identified in the BLM gene (encoding a RecQ helicase) in affected individuals, but the molecular mechanism and the structural basis of ..
  4. Kaneko H, Orii K, Matsui E, Shimozawa N, Fukao T, Matsumoto T, et al. BLM (the causative gene of Bloom syndrome) protein translocation into the nucleus by a nuclear localization signal. Biochem Biophys Res Commun. 1997;240:348-53 pubmed
    ..b>BLM was identified as the causative gene for BS, and BLM protein is homologous to DNA helicase...
  5. Bhattacharyya S, Keirsey J, Russell B, Kavecansky J, Lillard Wetherell K, Tahmaseb K, et al. Telomerase-associated protein 1, HSP90, and topoisomerase IIalpha associate directly with the BLM helicase in immortalized cells using ALT and modulate its helicase activity using telomeric DNA substrates. J Biol Chem. 2009;284:14966-77 pubmed publisher
    The BLM helicase associates with the telomere structural proteins TRF1 and TRF2 in immortalized cells using the alternative lengthening of telomere (ALT) pathways...
  6. Opresko P, Von Kobbe C, Laine J, Harrigan J, Hickson I, Bohr V. Telomere-binding protein TRF2 binds to and stimulates the Werner and Bloom syndrome helicases. J Biol Chem. 2002;277:41110-9 pubmed
    ..In vitro, TRF2 demonstrates high affinity for WRN and for another RecQ family member, the Bloom syndrome protein (BLM)...
  7. McDaniel L, Schultz R. Elevated sister chromatid exchange phenotype of Bloom syndrome cells is complemented by human chromosome 15. Proc Natl Acad Sci U S A. 1992;89:7968-72 pubmed
    ..Detailed characterization revealed this complementing element to be human chromosome 15. ..
  8. Nimonkar A, Ozsoy A, Genschel J, Modrich P, Kowalczykowski S. Human exonuclease 1 and BLM helicase interact to resect DNA and initiate DNA repair. Proc Natl Acad Sci U S A. 2008;105:16906-11 pubmed publisher
    ..Here, we establish that human BLM helicase, a member of the RecQ family, stimulates the nucleolytic activity of human exonuclease 1 (hExo1), a 5'--&..
  9. Stavropoulos D, Bradshaw P, Li X, Pasic I, Truong K, Ikura M, et al. The Bloom syndrome helicase BLM interacts with TRF2 in ALT cells and promotes telomeric DNA synthesis. Hum Mol Genet. 2002;11:3135-44 pubmed
    ..We find that endogenous BLM protein co-localizes with telomeric foci in ALT human cells but not telomerase positive immortal cell lines or ..

More Information

Publications88

  1. Pedrazzi G, Bachrati C, Selak N, Studer I, Petkovic M, Hickson I, et al. The Bloom's syndrome helicase interacts directly with the human DNA mismatch repair protein hMSH6. Biol Chem. 2003;384:1155-64 pubmed
    Bloom's syndrome (BS) is a rare genetic disorder characterised by genome instability and cancer susceptibility. BLM, the BS gene product, belongs to the highly-conserved RecQ family of DNA helicases...
  2. Opresko P, Mason P, Podell E, Lei M, Hickson I, Cech T, et al. POT1 stimulates RecQ helicases WRN and BLM to unwind telomeric DNA substrates. J Biol Chem. 2005;280:32069-80 pubmed
    Defects in human RecQ helicases WRN and BLM are responsible for the cancer-prone disorders Werner syndrome and Bloom syndrome...
  3. Ke Y, Huh J, Warrington R, Wu N, Leng M, Zhang J, et al. PICH and BLM limit histone association with anaphase centromeric DNA threads and promote their resolution. EMBO J. 2011;30:3309-21 pubmed publisher
    ..The SNF2 family helicase PICH (Plk1-interacting checkpoint helicase) and the BLM (the Bloom's syndrome protein) helicase decorate ultrafine histone-negative DNA threads that link the segregating ..
  4. Sengupta S, Robles A, Linke S, Sinogeeva N, Zhang R, Pedeux R, et al. Functional interaction between BLM helicase and 53BP1 in a Chk1-mediated pathway during S-phase arrest. J Cell Biol. 2004;166:801-13 pubmed
    ..Here, we show that BLM is an intermediate responder to stalled DNA replication forks...
  5. Rao V, Fan A, Meng L, Doe C, North P, Hickson I, et al. Phosphorylation of BLM, dissociation from topoisomerase IIIalpha, and colocalization with gamma-H2AX after topoisomerase I-induced replication damage. Mol Cell Biol. 2005;25:8925-37 pubmed
    ..b>BLM (Bloom's syndrome protein), a RecQ DNA helicase, and topoisomerase IIIalpha (Top3alpha) appear essential for the ..
  6. Wu L, Hickson I. The Bloom's syndrome helicase suppresses crossing over during homologous recombination. Nature. 2003;426:870-4 pubmed
    Mutations in BLM, which encodes a RecQ helicase, give rise to Bloom's syndrome, a disorder associated with cancer predisposition and genomic instability...
  7. Meetei A, Sechi S, Wallisch M, Yang D, Young M, Joenje H, et al. A multiprotein nuclear complex connects Fanconi anemia and Bloom syndrome. Mol Cell Biol. 2003;23:3417-26 pubmed
    ..of this disease, we isolated from human HeLa extracts three complexes containing the helicase defective in BS, BLM. Interestingly, one of the complexes, termed BRAFT, also contains five of the Fanconi anemia (FA) complementation ..
  8. Pedrazzi G, Perrera C, Blaser H, Kuster P, Marra G, Davies S, et al. Direct association of Bloom's syndrome gene product with the human mismatch repair protein MLH1. Nucleic Acids Res. 2001;29:4378-86 pubmed
    Bloom's syndrome (BS) is a rare genetic disorder characterised by genomic instability and cancer susceptibility. BLM, the gene mutated in BS, encodes a member of the RecQ family of DNA helicases...
  9. Franchitto A, Pichierri P. Bloom's syndrome protein is required for correct relocalization of RAD50/MRE11/NBS1 complex after replication fork arrest. J Cell Biol. 2002;157:19-30 pubmed
    ..with BS exhibit an elevated rate of somatic recombination and hypermutability, supporting a role for bleomycin (BLM) in the maintenance of genomic integrity...
  10. Mohaghegh P, Karow J, Brosh R, Bohr V, Hickson I. The Bloom's and Werner's syndrome proteins are DNA structure-specific helicases. Nucleic Acids Res. 2001;29:2843-9 pubmed
    b>BLM and WRN, the products of the Bloom's and Werner's syndrome genes, are members of the RecQ family of DNA helicases...
  11. Jiao R, Bachrati C, Pedrazzi G, Kuster P, Petkovic M, Li J, et al. Physical and functional interaction between the Bloom's syndrome gene product and the largest subunit of chromatin assembly factor 1. Mol Cell Biol. 2004;24:4710-9 pubmed
    ..The protein defective in BS, BLM, belongs to the RecQ family of DNA helicases...
  12. Janscak P, Garcia P, Hamburger F, Makuta Y, Shiraishi K, Imai Y, et al. Characterization and mutational analysis of the RecQ core of the bloom syndrome protein. J Mol Biol. 2003;330:29-42 pubmed
    b>Bloom syndrome protein forms an oligomeric ring structure and belongs to a group of DNA helicases showing extensive homology to the Escherichia coli DNA helicase RecQ, a suppressor of illegitimate recombination. After over-production in E...
  13. Gruber S, Ellis N, Scott K, Almog R, Kolachana P, Bonner J, et al. BLM heterozygosity and the risk of colorectal cancer. Science. 2002;297:2013 pubmed
  14. Lahkim Bennani Belhaj K, Rouzeau S, Buhagiar Labarchède G, Chabosseau P, Onclercq Delic R, Bayart E, et al. The Bloom syndrome protein limits the lethality associated with RAD51 deficiency. Mol Cancer Res. 2010;8:385-94 pubmed publisher
    Little is known about the functional interaction between the Bloom's syndrome protein (BLM) and the recombinase RAD51 within cells...
  15. Brosh R, Li J, Kenny M, Karow J, Cooper M, Kureekattil R, et al. Replication protein A physically interacts with the Bloom's syndrome protein and stimulates its helicase activity. J Biol Chem. 2000;275:23500-8 pubmed
    ..b>BLM, the gene defective in Bloom's syndrome, encodes a 159-kDa protein possessing DNA-stimulated ATPase and ATP-..
  16. Ding S, Yu J, Chen S, Hsu G, Kuo S, Lin Y, et al. Genetic variants of BLM interact with RAD51 to increase breast cancer susceptibility. Carcinogenesis. 2009;30:43-9 pubmed publisher
    ..Bloom (BLM) helicase directly interacts with RAD51 recombinase, which is involved in regulating HR, and it is thus of ..
  17. Naim V, Rosselli F. The FANC pathway and BLM collaborate during mitosis to prevent micro-nucleation and chromosome abnormalities. Nat Cell Biol. 2009;11:761-8 pubmed publisher
    ..The products of the 13 FANC genes (mutated in Fanconi anaemia), which constitute the 'FANC' pathway, and BLM (the RecQ helicase mutated in Bloom syndrome) are thought to collaborate during the S phase of the cell cycle, ..
  18. Davalos A, Kaminker P, Hansen R, Campisi J. ATR and ATM-dependent movement of BLM helicase during replication stress ensures optimal ATM activation and 53BP1 focus formation. Cell Cycle. 2004;3:1579-86 pubmed
    The BLM helicase, a deficiency that markedly increases cancer incidence in humans, is required for optimal repair during DNA replication...
  19. Yamagata K, Kato J, Shimamoto A, Goto M, Furuichi Y, Ikeda H. Bloom's and Werner's syndrome genes suppress hyperrecombination in yeast sgs1 mutant: implication for genomic instability in human diseases. Proc Natl Acad Sci U S A. 1998;95:8733-8 pubmed
    ..The genes responsible for these diseases, BLM and WRN, have been found to be homologs of Escherichia coli recQ and Saccharomyces cerevisiae SGS1 genes...
  20. Wu L, Chan K, Ralf C, Bernstein D, Garcia P, Bohr V, et al. The HRDC domain of BLM is required for the dissolution of double Holliday junctions. EMBO J. 2005;24:2679-87 pubmed
    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...
  21. German J, Sanz M, Ciocci S, Ye T, Ellis N. Syndrome-causing mutations of the BLM gene in persons in the Bloom's Syndrome Registry. Hum Mutat. 2007;28:743-53 pubmed
    Bloom syndrome (BS) is caused by homozygous or compound heterozygous mutations in the RecQ DNA helicase gene BLM. Since the molecular isolation of BLM, characterization of BS-causing mutations has been carried out systematically using ..
  22. Machwe A, Xiao L, Groden J, Matson S, Orren D. RecQ family members combine strand pairing and unwinding activities to catalyze strand exchange. J Biol Chem. 2005;280:23397-407 pubmed
    ..In this study, we show that three RecQ members (WRN, deficient in the Werner syndrome; BLM, deficient in the Bloom syndrome; and Drosophila melanogaster RecQ5b (dmRecQ5b)) possess a novel strand pairing ..
  23. Leng M, Chan D, Luo H, Zhu C, Qin J, Wang Y. MPS1-dependent mitotic BLM phosphorylation is important for chromosome stability. Proc Natl Acad Sci U S A. 2006;103:11485-90 pubmed
    ..BS cells exhibit elevated rates of sister chromatid exchange, chromosome breaks, and CIN. The BS gene product, BLM, is a member of the RecQ helicases that are required for maintenance of genome stability...
  24. Chabosseau P, Buhagiar Labarchède G, Onclercq Delic R, Lambert S, Debatisse M, Brison O, et al. Pyrimidine pool imbalance induced by BLM helicase deficiency contributes to genetic instability in Bloom syndrome. Nat Commun. 2011;2:368 pubmed publisher
    ..Bloom syndrome is caused by mutations in the BLM gene encoding a RecQ helicase...
  25. Davalos A, Campisi J. Bloom syndrome cells undergo p53-dependent apoptosis and delayed assembly of BRCA1 and NBS1 repair complexes at stalled replication forks. J Cell Biol. 2003;162:1197-209 pubmed
    ..b>BLM, the gene defective in BS, encodes a DNA helicase thought to participate in genomic maintenance...
  26. Bugreev D, Mazina O, Mazin A. Bloom syndrome helicase stimulates RAD51 DNA strand exchange activity through a novel mechanism. J Biol Chem. 2009;284:26349-59 pubmed publisher
    Loss or inactivation of BLM, a helicase of the RecQ family, causes Bloom syndrome, a genetic disorder with a strong predisposition to cancer...
  27. Davies S, North P, Dart A, Lakin N, Hickson I. Phosphorylation of the Bloom's syndrome helicase and its role in recovery from S-phase arrest. Mol Cell Biol. 2004;24:1279-91 pubmed
    Bloom's syndrome (BS) is a human genetic disorder associated with cancer predisposition. The BS gene product, BLM, is a member of the RecQ helicase family, which is required for the maintenance of genome stability in all organisms...
  28. Shimura T, Torres M, Martin M, Rao V, Pommier Y, Katsura M, et al. Bloom's syndrome helicase and Mus81 are required to induce transient double-strand DNA breaks in response to DNA replication stress. J Mol Biol. 2008;375:1152-64 pubmed
    ..a cell cycle checkpoint, but they did activate a process that required functional Bloom's syndrome-associated (BLM) helicase, Mus81 nuclease and ataxia telangiectasia mutated and Rad3-related (ATR) kinase to induce transient ..
  29. Orazio N, Naeger C, Karlseder J, Weitzman M. The adenovirus E1b55K/E4orf6 complex induces degradation of the Bloom helicase during infection. J Virol. 2011;85:1887-92 pubmed publisher
    ..We show that Bloom helicase (BLM) is degraded during adenovirus type 5 (Ad5) infection...
  30. Wang X, Tseng A, Ellis N, Spillare E, Linke S, Robles A, et al. Functional interaction of p53 and BLM DNA helicase in apoptosis. J Biol Chem. 2001;276:32948-55 pubmed
    The Bloom syndrome (BS) protein, BLM, is a member of the RecQ DNA helicase family that also includes the Werner syndrome protein, WRN. Inherited mutations in these proteins are associated with cancer predisposition of these patients...
  31. Grierson P, Lillard K, Behbehani G, Combs K, Bhattacharyya S, Acharya S, et al. BLM helicase facilitates RNA polymerase I-mediated ribosomal RNA transcription. Hum Mol Genet. 2012;21:1172-83 pubmed publisher
    ..The Bloom's syndrome helicase (BLM), mutations of which lead to BS, localizes to promyelocytic leukemia protein bodies and to the nucleolus of the ..
  32. Mendez Bermudez A, Hidalgo Bravo A, Cotton V, Gravani A, Jeyapalan J, Royle N. The roles of WRN and BLM RecQ helicases in the Alternative Lengthening of Telomeres. Nucleic Acids Res. 2012;40:10809-20 pubmed publisher
    ..Two RecQ helicase genes, BLM and WRN, play important roles in homologous recombination repair and they have been implicated in telomeric ..
  33. Ellis N, Groden J, Ye T, Straughen J, Lennon D, Ciocci S, et al. The Bloom's syndrome gene product is homologous to RecQ helicases. Cell. 1995;83:655-66 pubmed
    The Bloom's syndrome (BS) gene, BLM, plays an important role in the maintenance of genomic stability in somatic cells...
  34. Karow J, Newman R, Freemont P, Hickson I. Oligomeric ring structure of the Bloom's syndrome helicase. Curr Biol. 1999;9:597-600 pubmed
    ..The Bloom's syndrome gene product, BLM, belongs to the RecQ subfamily of DNA helicases and is required for the maintenance of genomic stability in human ..
  35. Woo L, Onel K, Ellis N. The broken genome: genetic and pharmacologic approaches to breaking DNA. Ann Med. 2007;39:208-18 pubmed
    ..This review discusses the roles of RecQ helicases in genomic stability, the interplay between RecQ helicases and topoisomerase 3s, and current and future prospects for targeting these interactions to develop novel anticancer therapies. ..
  36. Ababou M, Dumaire V, Lecluse Y, Amor Gueret M. Bloom's syndrome protein response to ultraviolet-C radiation and hydroxyurea-mediated DNA synthesis inhibition. Oncogene. 2002;21:2079-88 pubmed
    Bloom's syndrome (BS) arises through mutations in both copies of the BLM gene that encodes a RecQ 3'-5' DNA helicase...
  37. Barakat A, Ababou M, Onclercq R, Dutertre S, Chadli E, Hda N, et al. Identification of a novel BLM missense mutation (2706T>C) in a Moroccan patient with Bloom's syndrome. Hum Mutat. 2000;15:584-5 pubmed
  38. Sengupta S, Linke S, Pedeux R, Yang Q, Farnsworth J, Garfield S, et al. BLM helicase-dependent transport of p53 to sites of stalled DNA replication forks modulates homologous recombination. EMBO J. 2003;22:1210-22 pubmed
    ..It has been proposed that p53 and BLM help regulate these functions...
  39. Bischof O, Kim S, Irving J, Beresten S, Ellis N, Campisi J. Regulation and localization of the Bloom syndrome protein in response to DNA damage. J Cell Biol. 2001;153:367-80 pubmed
    ..b>BLM, the protein defective in BS, is a RecQ-like helicase, presumed to function in DNA replication, recombination, or ..
  40. Beamish H, Kedar P, Kaneko H, Chen P, Fukao T, Peng C, et al. Functional link between BLM defective in Bloom's syndrome and the ataxia-telangiectasia-mutated protein, ATM. J Biol Chem. 2002;277:30515-23 pubmed
    ..We report here direct interaction between BLM, mutated in Bloom's Syndrome (BS), and ATM, mutated is ataxia-telangiectasia, and we have mapped the sites of ..
  41. Suhasini A, Rawtani N, Wu Y, Sommers J, Sharma S, Mosedale G, et al. Interaction between the helicases genetically linked to Fanconi anemia group J and Bloom's syndrome. EMBO J. 2011;30:692-705 pubmed publisher
    ..BS and FA group J arise from mutations in the BLM and FANCJ genes, respectively, which encode DNA helicases...
  42. Wu L, Davies S, North P, Goulaouic H, Riou J, Turley H, et al. The Bloom's syndrome gene product interacts with topoisomerase III. J Biol Chem. 2000;275:9636-44 pubmed
    ..The Bloom's syndrome gene product, BLM, belongs to the RecQ family of DNA helicases, which also includes the human Werner's and Rothmund-Thomson syndrome ..
  43. Wu L, Davies S, Levitt N, Hickson I. Potential role for the BLM helicase in recombinational repair via a conserved interaction with RAD51. J Biol Chem. 2001;276:19375-81 pubmed
    ..The gene mutated in BS, BLM, encodes a member of the RecQ family of DNA helicases...
  44. Zhu J, Zhu S, Guzzo C, Ellis N, Sung K, Choi C, et al. Small ubiquitin-related modifier (SUMO) binding determines substrate recognition and paralog-selective SUMO modification. J Biol Chem. 2008;283:29405-15 pubmed publisher
    ..However, how different proteins are selectively modified by SUMO-1 and SUMO-2/3 is unknown. We demonstrate that BLM, the RecQ DNA helicase mutated in Bloom syndrome, is preferentially modified by SUMO-2/3 both in vitro and in vivo...
  45. Wu L, Hickson I. The Bloom's syndrome helicase stimulates the activity of human topoisomerase IIIalpha. Nucleic Acids Res. 2002;30:4823-9 pubmed
    ..The BS gene product, BLM, is a DNA helicase of the RecQ family that forms a complex in vitro and in vivo with topoisomerase IIIalpha...
  46. Wang Y, Cortez D, Yazdi P, Neff N, Elledge S, Qin J. BASC, a super complex of BRCA1-associated proteins involved in the recognition and repair of aberrant DNA structures. Genes Dev. 2000;14:927-39 pubmed
    ..This complex includes tumor suppressors and DNA damage repair proteins MSH2, MSH6, MLH1, ATM, BLM, and the RAD50-MRE11-NBS1 protein complex...
  47. Chan K, North P, Hickson I. BLM is required for faithful chromosome segregation and its localization defines a class of ultrafine anaphase bridges. EMBO J. 2007;26:3397-409 pubmed
    Mutations in BLM cause Bloom's syndrome, a disorder associated with cancer predisposition and chromosomal instability. We investigated whether BLM plays a role in ensuring the faithful chromosome segregation in human cells...
  48. Dutertre S, Ababou M, Onclercq R, Delic J, Chatton B, Jaulin C, et al. Cell cycle regulation of the endogenous wild type Bloom's syndrome DNA helicase. Oncogene. 2000;19:2731-8 pubmed
    ..BS arises through mutations in both alleles of the BLM gene which encodes a 3' - 5' DNA helicase identified as a member of the RecQ family...
  49. Davies S, North P, Hickson I. Role for BLM in replication-fork restart and suppression of origin firing after replicative stress. Nat Struct Mol Biol. 2007;14:677-9 pubmed
    Mutations in BLM give rise to Bloom's syndrome, a genetic disorder associated with cancer predisposition and chromosomal instability...
  50. Wang Y, Smith K, Waldman B, Waldman A. Depletion of the bloom syndrome helicase stimulates homology-dependent repair at double-strand breaks in human chromosomes. DNA Repair (Amst). 2011;10:416-26 pubmed publisher
    Mutation of BLM helicase causes Blooms syndrome, a disorder associated with genome instability, high levels of sister chromatid exchanges, and cancer predisposition...
  51. Rao V, Conti C, Guirouilh Barbat J, Nakamura A, Miao Z, Davies S, et al. Endogenous gamma-H2AX-ATM-Chk2 checkpoint activation in Bloom's syndrome helicase deficient cells is related to DNA replication arrested forks. Mol Cancer Res. 2007;5:713-24 pubmed
    The Bloom syndrome helicase (BLM) is critical for genomic stability. A defect in BLM activity results in the cancer-predisposing Bloom syndrome (BS)...
  52. Bugreev D, Yu X, Egelman E, Mazin A. Novel pro- and anti-recombination activities of the Bloom's syndrome helicase. Genes Dev. 2007;21:3085-94 pubmed
    ..The defining feature of BS is extreme genome instability. The gene mutated in Bloom's syndrome, BLM, encodes a DNA helicase (BLM) of the RecQ family...
  53. Johnson F, Lombard D, Neff N, Mastrangelo M, Dewolf W, Ellis N, et al. Association of the Bloom syndrome protein with topoisomerase IIIalpha in somatic and meiotic cells. Cancer Res. 2000;60:1162-7 pubmed
    Bloom syndrome (BS) is characterized by genomic instability and cancer susceptibility caused by defects in BLM, a DNA helicase of the RecQ-family (J. German and N. A. Ellis, The Genetic Basis of Human Cancer, pp. 301-316, 1998)...
  54. Larocque J, Jasin M. Mechanisms of recombination between diverged sequences in wild-type and BLM-deficient mouse and human cells. Mol Cell Biol. 2010;30:1887-97 pubmed publisher
    ..b>BLM-deficient mouse and human cells suppress homeologous recombination to a similar extent as wild-type cells, unlike ..
  55. Langland G, Elliott J, Li Y, Creaney J, Dixon K, Groden J. The BLM helicase is necessary for normal DNA double-strand break repair. Cancer Res. 2002;62:2766-70 pubmed
    ..This work demonstrates that BS cells, lacking the BLM helicase, process DSBs differently than normal cells and strongly suggests a role for the BLM helicase in aligning ..
  56. Karow J, Constantinou A, Li J, West S, Hickson I. The Bloom's syndrome gene product promotes branch migration of holliday junctions. Proc Natl Acad Sci U S A. 2000;97:6504-8 pubmed
    ..The gene mutated in BS, BLM, encodes a member of the RecQ family of DExH box DNA helicases, which also includes the Werner's syndrome gene ..
  57. Yang J, Bachrati C, Ou J, Hickson I, Brown G. Human topoisomerase IIIalpha is a single-stranded DNA decatenase that is stimulated by BLM and RMI1. J Biol Chem. 2010;285:21426-36 pubmed publisher
    Human topoisomerase IIIalpha is a type IA DNA topoisomerase that functions with BLM and RMI1 to resolve DNA replication and recombination intermediates...
  58. Nimonkar A, Genschel J, Kinoshita E, Polaczek P, Campbell J, Wyman C, et al. BLM-DNA2-RPA-MRN and EXO1-BLM-RPA-MRN constitute two DNA end resection machineries for human DNA break repair. Genes Dev. 2011;25:350-62 pubmed publisher
    ..We biochemically reconstituted DNA end resection using purified human proteins: Bloom helicase (BLM); DNA2 helicase/nuclease; Exonuclease 1 (EXO1); the complex comprising MRE11, RAD50, and NBS1 (MRN); and ..
  59. Popuri V, Bachrati C, Muzzolini L, Mosedale G, Costantini S, Giacomini E, et al. The Human RecQ helicases, BLM and RECQ1, display distinct DNA substrate specificities. J Biol Chem. 2008;283:17766-76 pubmed publisher
    ..Previous studies have shown that two human RecQ helicases, BLM and WRN, have very similar substrate specificities and preferentially unwind noncanonical DNA structures, such as ..
  60. Yang Q, Zhang R, Wang X, Linke S, Sengupta S, Hickson I, et al. The mismatch DNA repair heterodimer, hMSH2/6, regulates BLM helicase. Oncogene. 2004;23:3749-56 pubmed
    ..we show that the recombinant hMSH2/6 protein complex stimulated the ability of the Bloom's syndrome gene product, BLM, to process Holliday junctions in vitro, an activity that could also be regulated by p53...
  61. Wang F, Yang Y, Singh T, Busygina V, Guo R, Wan K, et al. Crystal structures of RMI1 and RMI2, two OB-fold regulatory subunits of the BLM complex. Structure. 2010;18:1159-70 pubmed publisher
    Mutations in BLM, a RecQ-like helicase, are linked to the autosomal recessive cancer-prone disorder Bloom's syndrome...
  62. Cleary S, Zhang W, Di Nicola N, Aronson M, Aube J, Steinman A, et al. Heterozygosity for the BLM(Ash) mutation and cancer risk. Cancer Res. 2003;63:1769-71 pubmed
    ..In contrast to the homozygous mutations of Bloom syndrome, heterozygous carriers of BLM mutations may be at increased risk for developing colorectal cancer...
  63. Eladad S, Ye T, Hu P, Leversha M, Beresten S, Matunis M, et al. Intra-nuclear trafficking of the BLM helicase to DNA damage-induced foci is regulated by SUMO modification. Hum Mol Genet. 2005;14:1351-65 pubmed
    The Bloom syndrome gene, BLM, encodes a RecQ DNA helicase that when absent from the cell results in genomic instability and cancer predisposition...
  64. Hu P, Beresten S, van Brabant A, Ye T, Pandolfi P, Johnson F, et al. Evidence for BLM and Topoisomerase IIIalpha interaction in genomic stability. Hum Mol Genet. 2001;10:1287-98 pubmed
    ..The primary cause of the genomic instability is mutation at BLM, which encodes a DNA helicase of the RecQ family...
  65. Foucault F, Vaury C, Barakat A, Thibout D, Planchon P, Jaulin C, et al. Characterization of a new BLM mutation associated with a topoisomerase II alpha defect in a patient with Bloom's syndrome. Hum Mol Genet. 1997;6:1427-34 pubmed
    ..recessive disorder associated with an increased risk of malignancy, arises through mutations in both alleles of the BLM gene, which was recently identified as a member of the RecQ helicase family...
  66. Karow J, Chakraverty R, Hickson I. The Bloom's syndrome gene product is a 3'-5' DNA helicase. J Biol Chem. 1997;272:30611-4 pubmed
    ..The gene mutated in BS, BLM, encodes a protein containing seven "signature" motifs conserved in a wide range of DNA and RNA helicases...
  67. Lillard Wetherell K, Machwe A, Langland G, Combs K, Behbehani G, Schonberg S, et al. Association and regulation of the BLM helicase by the telomere proteins TRF1 and TRF2. Hum Mol Genet. 2004;13:1919-32 pubmed
    In addition to increased DNA-strand exchange, a cytogenetic feature of cells lacking the RecQ-like BLM helicase is a tendency for telomeres to associate...
  68. Srivastava V, Modi P, Tripathi V, Mudgal R, De S, Sengupta S. BLM helicase stimulates the ATPase and chromatin-remodeling activities of RAD54. J Cell Sci. 2009;122:3093-103 pubmed publisher
    Mutation of BLM helicase results in the autosomal recessive disorder Bloom syndrome (BS). Patients with BS exhibit hyper-recombination and are prone to almost all forms of cancer...
  69. Von Kobbe C, Karmakar P, Dawut L, Opresko P, Zeng X, Brosh R, et al. Colocalization, physical, and functional interaction between Werner and Bloom syndrome proteins. J Biol Chem. 2002;277:22035-44 pubmed
    ..These include the WRN and BLM gene products that are defective in Werner and Bloom syndromes, disorders which share many phenotypic and cellular ..
  70. Gyimesi M, Sarlós K, Kovacs M. Processive translocation mechanism of the human Bloom's syndrome helicase along single-stranded DNA. Nucleic Acids Res. 2010;38:4404-14 pubmed publisher
    b>BLM, one of the human RecQ helicases, plays a fundamental role in homologous recombination-based error-free DNA repair pathways, which require its translocation and DNA unwinding activities...
  71. Xu D, Muniandy P, Leo E, Yin J, Thangavel S, Shen X, et al. Rif1 provides a new DNA-binding interface for the Bloom syndrome complex to maintain normal replication. EMBO J. 2010;29:3140-55 pubmed publisher
    b>BLM, the helicase defective in Bloom syndrome, is part of a multiprotein complex that protects genome stability...
  72. Bergeron K, Murphy E, Brown L, Almeida K. Critical interaction domains between bloom syndrome protein and RAD51. Protein J. 2011;30:1-8 pubmed publisher
    ..Bloom Syndrome, a rare disorder characterized by a predisposition to cancer, is caused by mutations of the BLM gene...
  73. Raynard S, Bussen W, Sung P. A double Holliday junction dissolvasome comprising BLM, topoisomerase IIIalpha, and BLAP75. J Biol Chem. 2006;281:13861-4 pubmed
    ..b>BLM, the RecQ-like helicase mutated in BS, is found in several cellular protein complexes, all of which contain ..
  74. Raynard S, Zhao W, Bussen W, Lu L, Ding Y, Busygina V, et al. Functional role of BLAP75 in BLM-topoisomerase IIIalpha-dependent holliday junction processing. J Biol Chem. 2008;283:15701-8 pubmed publisher
    The BLAP75 protein combines with the BLM helicase and topoisomerase (Topo) IIIalpha to form an evolutionarily conserved complex, termed the BTB complex, that functions to regulate homologous recombination...
  75. Langland G, Kordich J, Creaney J, Goss K, Lillard Wetherell K, Bebenek K, et al. The Bloom's syndrome protein (BLM) interacts with MLH1 but is not required for DNA mismatch repair. J Biol Chem. 2001;276:30031-5 pubmed
    ..BS is the consequence of mutations that lead to loss of function of BLM, a gene encoding a helicase with homology to the RecQ helicase family...
  76. Wu L, Bachrati C, Ou J, Xu C, Yin J, Chang M, et al. BLAP75/RMI1 promotes the BLM-dependent dissolution of homologous recombination intermediates. Proc Natl Acad Sci U S A. 2006;103:4068-73 pubmed
    b>BLM encodes a member of the highly conserved RecQ DNA helicase family, which is essential for the maintenance of genome stability. Homozygous inactivation of BLM gives rise to the cancer predisposition disorder Bloom's syndrome...
  77. Yin J, Sobeck A, Xu C, Meetei A, Hoatlin M, Li L, et al. BLAP75, an essential component of Bloom's syndrome protein complexes that maintain genome integrity. EMBO J. 2005;24:1465-76 pubmed
    ..We have previously purified three complexes containing BLM, the helicase mutated in this disease...
  78. Ralf C, Hickson I, Wu L. The Bloom's syndrome helicase can promote the regression of a model replication fork. J Biol Chem. 2006;281:22839-46 pubmed
    Homozygous inactivation of BLM gives rise to Bloom's syndrome, a disorder associated with genomic instability and cancer predisposition...
  79. Sharma S, Sommers J, Wu L, Bohr V, Hickson I, Brosh R. Stimulation of flap endonuclease-1 by the Bloom's syndrome protein. J Biol Chem. 2004;279:9847-56 pubmed
    ..is a temporal delay in the maturation of DNA replication intermediates, the precise role of the BS gene product, BLM, in DNA metabolism remains elusive...