Prof Brian Bigger PhD

Professor of Cell and Gene Therapy

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Publications

  1. 1999
  2. Published

    Tipping the scales in favour of mitochondrial gene therapy

    Bigger, B., Collombet, J. M. & Coutelle, C., Dec 1999, In: Gene Therapy. 6, 12, p. 1909-1910 1 p.

    Research output: Contribution to journalArticlepeer-review

  3. 2000
  4. Published

    Introduction of chloramphenicol resistance into the modified mouse mitochondrial genome: Cloning of unstable sequences by passage through yeast

    Bigger, B., Tolmachov, O., Collombet, J. M. & Coutelle, C., 15 Jan 2000, In: Analytical Biochemistry. 277, 2, p. 236-242 6 p.

    Research output: Contribution to journalArticlepeer-review

  5. 2001
  6. Published

    Gentherapie der zystischen fibrose: Eine analyse der bisherigen klinischen studien

    Coutelle, C. & Bigger, B., 2001, In: Internist. 42, 10, p. 1346-1356 10 p.

    Research output: Contribution to journalArticlepeer-review

  7. Published

    Perspectives on gene therapy for cystic fibrosis airway disease

    Bigger, B. & Coutelle, C., 2001, In: BioDrugs. 15, 9, p. 615-634 19 p.

    Research output: Contribution to journalArticlepeer-review

  8. Published

    An araC-controlled Bacterial cre Expression System to Produce DNA Minicircle Vectors for Nuclear and Mitochondrial Gene Therapy

    Bigger, B. W., Tolmachov, O., Collombet, J. M., Fragkos, M., Palaszewski, I. & Coutelle, C., 22 Jun 2001, In: Journal of Biological Chemistry. 276, 25, p. 23018-23027 9 p.

    Research output: Contribution to journalArticlepeer-review

  9. 2003
  10. Published

    Gene Therapy for Mitochondrial DNA Disorders

    Bigger, B., Lightowlers, R. N., Taylor, R. W., Turnbull, D. & Holt, IJ. (ed.), 2003, Genetics of Mitochondrial Diseases. Oxford: Oxford University Press

    Research output: Chapter in Book/Report/Conference proceedingChapter

  11. Published

    Long-term transgene expression by administration of a lentivirus-based vector to the fetal circulation of immuno-competent mice

    Waddington, S. N., Mitrophanous, K. A., Ellard, F. M., Buckley, S. M. K., Nivsarkar, M., Lawrence, L., Cook, H. T., Al-Allaf, F., Bigger, B., Kingsman, S. M., Coutelle, C. & Themis, M., Aug 2003, In: Gene Therapy. 10, 15, p. 1234-1240 6 p.

    Research output: Contribution to journalArticlepeer-review

  12. 2004
  13. Published

    Hepatic stem cells: From inside and outside the liver?

    Alison, M. R., Vig, P., Russo, F., Bigger, B. W., Amofah, E., Themis, M. & Forbes, S., Feb 2004, In: Cell Proliferation. 37, 1, p. 1-21 20 p.

    Research output: Contribution to journalArticlepeer-review

  14. Published

    Development of a Self-assembling Nuclear Targeting Vector System Based on the Tetracycline Repressor Protein

    Vaysse, L., Harbottle, R., Bigger, B., Bergau, A., Tolmachov, O. & Coutelle, C., 13 Feb 2004, In: Journal of Biological Chemistry. 279, 7, p. 5555-5564 9 p.

    Research output: Contribution to journalArticlepeer-review

  15. Published

    Highly efficient EIAV-mediated in utero gene transfer and expression in the major muscle groups affected by Duchenne muscular dystrophy

    Bigger, B., Gregory, L. G., Waddington, S. N., Holder, M. V., Mitrophanous, K. A., Buckley, S. M. K., Mosley, K. L., Bigger, B. W., Ellard, F. M., Walmsley, L. E., Lawrence, L., Al-Allaf, F., Kingsman, S., Coutelle, C. & Themis, M., Jul 2004, In: Gene Therapy. 11, 14, p. 1117-1125 8 p.

    Research output: Contribution to journalArticlepeer-review

  16. Published

    Permanent phenotypic correction of hemophilia B in immunocompetent mice by prenatal gene therapy

    Waddington, S. N., Nivsarkar, M. S., Mistry, A. R., Buckley, S. M. K., Kemball-Cook, G., Mosley, K. L., Mitrophanous, K., Radcliffe, P., Holder, M. V., Brittan, M., Georgiadis, A., Al-Allaf, F., Bigger, B. W., Gregory, L. G., Terence Cook, H., Ali, R. R., Thrasher, A., Tuddenham, E. G. D., Themis, M. & Coutelle, C., 1 Nov 2004, In: Blood. 104, 9, p. 2714-2721 7 p.

    Research output: Contribution to journalArticlepeer-review

  17. 2005
  18. Published

    Minimised, CpG-depleted and methylated DNA vectors: Towards perfection in non-viral gene therapy

    Bigger, B. W., Tolmachov, O., Harbottle, R., Coutelle, C. & Schleef, M. (ed.), 2005, DNA-Pharmaceuticals: Formulation and delivery in gene therapy, DNA vaccination and immunotherapy. Cambridge, UK: John Wiley & Sons Ltd

    Research output: Chapter in Book/Report/Conference proceedingChapter

  19. Published

    Comparison of HIV- and EIAV-based vectors on their efficiency in transducing murine and human hematopoietic repopulating cells

    Siapati, E. K., Bigger, B. W., Miskin, J., Chipchase, D., Parsley, K. L., Mitrophanous, K., Themis, M., Thrasher, A. J. & Bonnet, D., Sep 2005, In: Molecular Therapy. 12, 3, p. 537-546 9 p.

    Research output: Contribution to journalArticlepeer-review

  20. Published

    Oncogenesis following delivery of a nonprimate lentiviral gene therapy vector to fetal and neonatal mice

    Themis, M., Waddington, S. N., Schmidt, M., von Kalle, C., Wang, Y., Al-Allaf, F., Gregory, L. G., Nivsarkar, M., Themis, M., Holder, M. V., Buckley, S. M. K., Dighe, N., Ruthe, A. T., Mistry, A., Bigger, B., Rahim, A., Nguyen, T. H., Trono, D., Thrasher, A. J. & Coutelle, C., Oct 2005, In: Molecular Therapy. 12, 4, p. 763-771 8 p.

    Research output: Contribution to journalArticlepeer-review

  21. 2006
  22. Published

    Permanent partial phenotypic correction and tolerance in a mouse model of hemophilia B by stem cell gene delivery of human factor IX

    Bigger, B., Siapati, E. K., Mistry, A., Waddington, S. N., Nivsarkar, M. S., Jacobs, L., Perrett, R., Holder, M. V., Ridler, C., Kemball-Cook, G., Ali, R. R., Forbes, S. J., Coutelle, C., Wright, N., Alison, M., Thrasher, A. J., Bonnet, D. & Themis, M., Jan 2006, In: Gene Therapy. 13, 2, p. 117-126 9 p.

    Research output: Contribution to journalArticlepeer-review

  23. Published

    RecET driven chromosomal gene targeting to generate a RecA deficient Escherichia coli strain for Cre mediated production of minicircle DNA

    Tolmachov, O., Palaszewski, I., Bigger, B. & Coutelle, C., 10 Mar 2006, In: BMC Biotechnology. 6, 17.

    Research output: Contribution to journalArticlepeer-review

  24. Published

    The Bone Marrow Functionally Contributes to Liver Fibrosis

    Russo, F. P., Alison, M. R., Bigger, B. W., Amofah, E., Florou, A., Amin, F., Bou-Gharios, G., Jeffery, R., Iredale, J. P. & Forbes, S. J., Jun 2006, In: Gastroenterology. 130, 6, p. 1807-1821 14 p.

    Research output: Contribution to journalArticlepeer-review

  25. 2007
  26. Published

    Murine leukemia following irradiation conditioning for transplantation of lentivirally-modified hematopoietic stem cells

    Siapati, E. K., Bigger, B. W., Kashofer, K., Themis, M., Thrasher, A. J. & Bonnet, D., Apr 2007, In: European journal of haematology. 78, 4, p. 303-313 10 p.

    Research output: Contribution to journalArticlepeer-review

  27. Published

    Xenogenic transfer of isolated murine mitochondria into human ρ0 cells can improve respiratory function

    Katrangi, E., D'Souza, G., Boddapati, S. V., Kulawiec, M., Singh, K. K., Bigger, B. & Weissig, V., 1 Dec 2007, In: Rejuvenation Research. 10, 4, p. 561-570 9 p.

    Research output: Contribution to journalArticlepeer-review

  28. 2009
  29. Published

    Improved Metabolic Correction in Patients with Lysosomal Storage Disease Treated with Hematopoietic Stem Cell Transplant Compared with Enzyme Replacement Therapy

    Wynn, R. F., Wraith, J. E., Mercer, J., O'Meara, A., Tylee, K., Thornley, M., Church, H. J. & Bigger, B. W., Apr 2009, In: Journal Of Pediatrics. 154, 4, p. 609-611 2 p.

    Research output: Contribution to journalArticlepeer-review

  30. Published

    Cellular therapy of lysosomal storage disorders: Current status and future prospects

    Wynn, R. F., Stubbs, M., Ozyilmaz, N., Wraith, J. E. & Bigger, B., Aug 2009, In: Current Pediatric Reviews. 5, 3, p. 147-159 12 p.

    Research output: Contribution to journalArticlepeer-review

  31. Published

    Genistein reduces lysosomal storage in peripheral tissues of mucopolysaccharide IIIB mice

    Malinowska, M., Wilkinson, F. L., Bennett, W., Langford-Smith, K. J., O'Leary, H. A., Jakobkiewicz-Banecka, J., Wynn, R., Wraith, J. E., Wegrzyn, G. & Bigger, B. W., Nov 2009, In: Molecular genetics and metabolism. 98, 3, p. 235-242 7 p.

    Research output: Contribution to journalArticlepeer-review

  32. 2010
  33. Published

    Genistein improves neuropathology and corrects behaviour in a mouse model of neurodegenerative metabolic disease

    Malinowska, M., Wilkinson, F. L., Langford-Smith, K. J., Langford-Smith, A., Brown, J. R., Crawford, B. E., Vanier, M. T., Grynkiewicz, G., Wynn, R. F., Ed Wraith, J., Wegrzyn, G. & Bigger, B. W., 2010, In: PLoS ONE. 5, 12, e14192.

    Research output: Contribution to journalArticlepeer-review

  34. Published

    Successful allogeneic bone marrow transplant for Niemann-Pick disease type C2 is likely to be associated with a severe 'graft versus substrate' effect

    Bonney, D. K., O'Meara, A., Shabani, A., Imrie, J., Bigger, B. W., Jones, S., Wraith, J. E. & Wynn, R. F., 2010, In: Journal of Inherited Metabolic Disease. 33, 3, p. S171-S173

    Research output: Contribution to journalArticlepeer-review

  35. Published

    Immune activation or immunomodulation in the brains of MPS IIIB mice? Commentary on "innate and adaptive immune activation in the brain of MPS IIIB mouse model"

    Archer, L. D., Langford, K., Bigger, B. & Fildes, J. E., 1 Feb 2010, In: Journal of Neuroscience Research. 88, 2, p. 233

    Research output: Contribution to journalArticlepeer-review

  36. Published

    Evaluation of heparin cofactor II-thrombin complex as a biomarker on blood spots from mucopolysaccharidosis I, IIIA and IIIB mice

    Langford-Smith, K., Arasaradnam, M., Wraith, J. E., Wynn, R. & Bigger, B. W., Mar 2010, In: Molecular genetics and metabolism. 99, 3, p. 269-274 5 p.

    Research output: Contribution to journalArticlepeer-review

  37. Published

    Circadian rhythm and suprachiasmatic nucleus alterations in the mouse model of mucopolysaccharidosis IIIB

    Canal, M. M., Wilkinson, F. L., Cooper, J. D., Ed Wraith, J., Wynn, R. & Bigger, B. W., 19 Jun 2010, In: Behavioural brain research. 209, 2, p. 212-220 8 p.

    Research output: Contribution to journalArticlepeer-review

  38. 2011
  39. Published

    Heparin cofactor II-thrombin complex and dermatan sulphate:chondroitin sulphate ratio are biomarkers of short- and long-term treatment effects in mucopolysaccharide diseases

    Langford-Smith, K. J., Mercer, J., Petty, J., Tylee, K., Church, H., Roberts, J., Moss, G., Jones, S., Wynn, R., Wraith, J. E. & Bigger, B. W., Apr 2011, In: Journal of Inherited Metabolic Disease. 34, 2, p. 499-508 9 p.

    Research output: Contribution to journalArticlepeer-review

  40. Published

    Chronic norovirus infection in pediatric hematopoietic stem cell transplant recipients: A cause of prolonged intestinal failure requiring intensive nutritional support

    Saif, M. A., Bonney, D. K., Bigger, B., Forsythe, L., Williams, N., Page, J., Babiker, Z. O., Guiver, M., Turner, A. J., Hughes, S. & Wynn, R. F., Aug 2011, In: Pediatric transplantation. 15, 5, p. 505-509 4 p.

    Research output: Contribution to journalArticlepeer-review

  41. Published

    Hyperactive behaviour in the mouse model of mucopolysaccharidosis iiib in the open field and home cage environments

    Langford-Smith, A., Malinowska, M., Langford-Smith, K. J., Wegrzyn, G., Jones, S., Wynn, R., Wraith, J. E., Wilkinson, F. L. & Bigger, B. W., Aug 2011, In: Genes, Brain and Behavior. 10, 6, p. 673-682 9 p.

    Research output: Contribution to journalArticlepeer-review

  42. Published

    Female mucopolysaccharidosis IIIA mice exhibit hyperactivity and a reduced sense of danger in the open field test

    Langford-Smith, A., Langford-Smith, K. J., Jones, S. A., Wynn, R. F., Wraith, J. E., Wilkinson, F. L. & Bigger, B. W., 18 Oct 2011, In: PLoS ONE. 6, 10, e25717.

    Research output: Contribution to journalArticlepeer-review

  43. Published

    Mucopolysaccharidosis type I, unique structure of accumulated heparan sulfate and increased N-sulfotransferase activity in mice lacking α-L-iduronidase

    Holley, R. J., Deligny, A., Wei, W., Watson, H. A., Niñonuevo, M. R., Dagälv, A., Leary, J. A., Bigger, B. W., Kjellén, L. & Merry, C. L. R., 28 Oct 2011, In: Journal of Biological Chemistry. 286, 43, p. 37515-37524 9 p.

    Research output: Contribution to journalArticlepeer-review

  44. Published

    Trial and error: How the unclonable human mitochondrial genome was cloned in yeast

    Bigger, B. W., Liao, A. Y., Sergijenko, A. & Coutelle, C., Nov 2011, In: Pharmaceutical Research. 28, 11, p. 2863-2870 7 p.

    Research output: Contribution to journalArticlepeer-review

  45. 2012
  46. Published

    Neuropathology in mouse models of mucopolysaccharidosis type I, IIIA and IIIB

    Wilkinson, F. L., Holley, R. J., Langford-Smith, K. J., Badrinath, S., Liao, A., Langford-Smith, A., Cooper, J. D., Jones, S. A., Wraith, J. E., Wynn, R. F., Merry, C. L. R. & Bigger, B. W., 27 Apr 2012, In: P L o S One. 7, 4, e35787.

    Research output: Contribution to journalArticlepeer-review

  47. Published

    Hematopoietic stem cell and gene therapy corrects primary neuropathology and behavior in mucopolysaccharidosis IIIA mice

    Langford-Smith, A., Wilkinson, F. L., Langford-Smith, K. J., Holley, R. J., Sergijenko, A., Howe, S. J., Bennett, W. R., Jones, S. A., Wraith, J. E., Merry, C. L. R., Wynn, R. F. & Bigger, B. W., Aug 2012, In: Molecular Therapy. 20, 8, p. 1610-1621 11 p.

    Research output: Contribution to journalArticlepeer-review

  48. Published

    Hematopoietic stem cell transplantation improves the high incidence of neutralizing allo-antibodies observed in Hurler's syndrome after pharmacological enzyme replacement therapy

    Saif, M., Bigger, B., Brookes, K., Mercer, J., Tylee, K. L., Church, H. J., Bonney, D. K., Jones, S., Ed Wraith, J. & Wynn, R. F., 1 Sep 2012, In: Haematologica. 97, 9, p. 1320-1328 8 p.

    Research output: Contribution to journalArticlepeer-review

  49. 2013
  50. Published

    The fetal mouse is a sensitive genotoxicity model that exposes lentiviral-associated mutagenesis resulting in liver oncogenesis

    Nowrouzi, A., Cheung, W. T., Li, T., Zhang, X., Arens, A., Paruzynski, A., Waddington, S. N., Osejindu, E., Reja, S., Von Kalle, C., Wang, Y., Al-Allaf, F., Gregory, L., Themes, M., Holder, M., Dighe, N., Ruthe, A., Buckley, S. M. K., Bigger, B., Montini, E. & 7 others, Thrasher, A. J., Andrews, R., Roberts, T. P., Newbold, R. F., Coutelle, C., Schmidt, M. & Themis, M., Feb 2013, In: Molecular Therapy. 21, 2, p. 324-337 13 p.

    Research output: Contribution to journalArticlepeer-review

  51. Published

    Characterisation of the T cell and dendritic cell repertoire in a murine model of mucopolysaccharidosis i (MPS I)

    Archer, L. D., Langford-Smith, K. J., Critchley, W. R., Bigger, B. W. & Fildes, J. E., Mar 2013, In: Journal of Inherited Metabolic Disease. 36, 2, p. 257-262 5 p.

    Research output: Contribution to journalArticlepeer-review

  52. Published

    Parental social support, coping strategies, resilience factors, stress, anxiety and depression levels in parents of children with MPS III (Sanfilippo syndrome) or children with intellectual disabilities (ID)

    Grant, S., Cross, E., Wraith, J. E., Jones, S., Mahon, L., Lomax, M., Bigger, B. & Hare, D., Mar 2013, In: Journal of Inherited Metabolic Disease. 36, 2, p. 281-291 10 p.

    Research output: Contribution to journalArticlepeer-review

  53. Published

    Plasma and urinary levels of dermatan sulfate and heparan sulfate derived disaccharides after long-term enzyme replacement therapy (ERT) in MPS I: correlation with the timing of ERT and with total urinary excretion of glycosaminoglycans

    De Ru, M. H., Van Der Tol, L., Van Vlies, N., Bigger, B. W., Hollak, C. E. M., Ijlst, L., Kulik, W., Van Lenthe, H., Saif, M., Wagemans, T., Van Der Wal, W. M., Wanders, R. J. & Wijburg, F. A., Mar 2013, In: Journal of inherited metabolic disorders. 36, 2, p. 247-255 8 p.

    Research output: Contribution to journalArticlepeer-review

  54. Published

    Busulfan conditioning enhances engraftment of hematopoietic donor-derived cells in the brain compared with irradiation

    Wilkinson, F. L., Sergijenko, A., Langford-Smith, K. J., Malinowska, M., Wynn, R. F. & Bigger, B. W., Apr 2013, In: Molecular Therapy. 21, 4, p. 868-876 8 p.

    Research output: Contribution to journalArticlepeer-review

  55. Published

    Analytical strategies for characterization of oxysterol lipidomes: Liver X receptor ligands in plasma

    Griffiths, W. J., Crick, P. J., Wang, Y., Ogundare, M., Tuschl, K., Morris, A. A., Bigger, B. W., Clayton, P. T. & Wang, Y., Jun 2013, In: Free Radical Biology and Medicine. 59, p. 69-84 15 p.

    Research output: Contribution to journalArticlepeer-review

  56. Published

    Central and haematopoietic interleukin-1 both contribute to ischaemic brain injury in mice

    Denes, A., Wilkinson, F., Bigger, B., Chu, M., Rothwell, N. J. & Allan, S. M., Jul 2013, In: DMM Disease Models and Mechanisms. 6, 4, p. 1043-1048 5 p.

    Research output: Contribution to journalArticlepeer-review

  57. Published

    Myeloid/Microglial driven autologous hematopoietic stem cell gene therapy corrects a neuronopathic lysosomal disease

    Sergijenko, A., Langford-Smith, A., Liao, A. Y., Pickford, C. E., McDermott, J., Nowinski, G., Langford-Smith, K. J., Merry, C. L., Jones, S. A., Wraith, J. E., Wynn, R. F., Wilkinson, F. L. & Bigger, B. W., Oct 2013, In: Molecular Therapy. 21, 10, p. 1938-1949 11 p.

    Research output: Contribution to journalArticlepeer-review

  58. Published

    Signal One and Two Blockade Are Both Critical for Non-Myeloablative Murine HSCT across a Major Histocompatibility Complex Barrier

    Langford-Smith, K. J., Sandiford, Z., Langford-Smith, A., Wilkinson, F. L., Jones, S. A., Wraith, J. E., Wynn, R. F. & Bigger, B. W., 17 Oct 2013, In: PLoS ONE. 8, 10, e77632.

    Research output: Contribution to journalArticlepeer-review

  59. 2014
  60. Published

    An investigation of the middle and late behavioural phenotypes of Mucopolysaccharidosis Type-III.

    Cross, E. M., Grant, S., Jones, S., Bigger, B. W., Wraith, J. E., Mahon, L. V., Lomax, M. & Hare, D. J., 2014, In: Journal of neurodevelopmental disorders. 6, 1

    Research output: Contribution to journalArticlepeer-review

  61. Published

    Novel approaches and mechanisms in hematopoietic stem cell gene therapy

    Bigger, B. W. & Wynn, R. F., 2014, In: Discovery Medicine. 17, 94

    Research output: Contribution to journalArticlepeer-review

  62. Published

    Mucopolysaccharide diseases: A complex interplay between neuroinflammation, microglial activation and adaptive immunity

    Archer, L. D., Langford-Smith, K. J., Bigger, B. W. & Fildes, J. E., Jan 2014, In: Journal of Inherited Metabolic Disease. 37, 1, p. 1-12 11 p.

    Research output: Contribution to journalArticlepeer-review

  63. Published

    Assessment of sleep in children with mucopolysaccharidosis type III

    Mahon, L. V., Lomax, M., Grant, S., Cross, E., Hare, D. J., Wraith, J. E., Jones, S., Bigger, B., Langford-Smith, K. & Canal, M., 4 Feb 2014, In: PLoS ONE. 9, 2, e84128.

    Research output: Contribution to journalArticlepeer-review

  64. Published

    Heparan sulfate inhibits hematopoietic stem and progenitor cell migration and engraftment in mucopolysaccharidosis I.

    Watson, H. A., Holley, R. J., Langford-Smith, K. J., Wilkinson, F. L., van Kuppevelt, T. H., Wynn, R. F., Wraith, J. E., Merry, C. L. R. & Bigger, B. W., 26 Dec 2014, In: The Journal of biological chemistry. 289, 52, p. 36194-36203 9 p.

    Research output: Contribution to journalArticlepeer-review

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