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

  65. 2015
  66. Published

    Actigraphic investigation of circadian rhythm functioning and activity levels in children with mucopolysaccharidosis type III (Sanfilippo syndrome).

    Mumford, R. A., Mahon, L. V., Jones, S., Bigger, B., Canal, M. & Hare, D. J., 2015, In: Journal of neurodevelopmental disorders. 7, 1

    Research output: Contribution to journalArticlepeer-review

  67. Published

    Sleep disordered breathing in mucopolysaccharidosis I: a multivariate analysis of patient, therapeutic and metabolic correlators modifying long term clinical outcome.

    Pal, A. R., Langereis, E. J., Saif, M. A., Mercer, J., Church, H. J., Tylee, K. L., Wynn, R. F., Wijburg, F. A., Jones, S. A., Bruce, I. A. & Bigger, B. W., 2015, In: Orphanet Journal of Rare Diseases. 10

    Research output: Contribution to journalArticlepeer-review

  68. Published

    Is it congenital or acquired von Willebrands disease?

    Saif, MA., Thachil, J., Brown, R., Bigger, BW., Wynn, RF., Nash, M. & Hay, CR., Jan 2015, In: Haemophilia. 21, 1, p. e113-e115

    Research output: Contribution to journalArticlepeer-review

  69. Published

    Neuroinflammation, mitochondrial defects and neurodegeneration in mucopolysaccharidosis III type C mouse model

    Martins, C., Hůlková, H., Dridi, L., Dormoy-Raclet, V., Grigoryeva, L., Choi, Y., Langford-Smith, A., Wilkinson, F. L., Ohmi, K., DiCristo, G., Hamel, E., Ausseil, J., Cheillan, D., Moreau, A., Svobodová, E., Hájková, Z., Tesařová, M., Hansíková, H., Bigger, B. W., Hrebícek, M. & 1 others, Pshezhetsky, A. V., 7 Jan 2015, In: Brain. 138, Pt 2, p. 336-355

    Research output: Contribution to journalArticlepeer-review

  70. Published

    Biomarker responses correlate with antibody status in mucopolysaccharidosis type I patients on long-term enzyme replacement therapy.

    Langereis, E. J., van Vlies, N., Church, H. J., Geskus, R. B., Hollak, C. E. M., Jones, S. A., Kulik, W., van Lenthe, H., Mercer, J., Schreider, L., Tylee, K. L., Wagemans, T., Wijburg, F. A. & Bigger, B. W., Feb 2015, In: Molecular genetics and metabolism. 114, 2

    Research output: Contribution to journalArticlepeer-review

  71. Published

    Quantitative charge-tags for sterol and oxysterol analysis.

    Crick, P. J., William Bentley, T., Abdel-Khalik, J., Matthews, I., Clayton, P. T., Morris, A. A., Bigger, B. W., Zerbinati, C., Tritapepe, L., Iuliano, L., Wang, Y. & Griffiths, W. J., Feb 2015, In: Clinical Chemistry. 61, 2

    Research output: Contribution to journalArticlepeer-review

  72. Published

    In vivo T-cell depletion using alemtuzumab in family and unrelated donor transplantation for pediatric non-malignant disease achieves engraftment with low incidence of graft vs. host disease.

    Saif, M. A., Borrill, R., Bigger, B. W., Lee, H., Logan, A., Poulton, K., Hughes, S., Turner, A. J., Bonney, D. K. & Wynn, R. F., Mar 2015, In: Pediatric transplantation. 19, 2

    Research output: Contribution to journalArticlepeer-review

  73. Published

    The role of antibodies in enzyme treatments and therapeutic strategies.

    Bigger, B. W., Saif, M. & Linthorst, G. E., Mar 2015, In: Best practice & research. Clinical endocrinology & metabolism. 29, 2

    Research output: Contribution to journalArticlepeer-review

  74. 2016
  75. Published

    The impact of the immune system on the safety and efficiency of enzyme replacement therapy in lysosomal storage disorders

    Broomfield, A., Jones, S. A., Hughes, S. M. & Bigger, B. W., 16 Feb 2016, In: Journal of Inherited Metabolic Disease. 39, 4, p. 499-512 14 p.

    Research output: Contribution to journalArticlepeer-review

  76. Published

    Hematopoietic stem cell transplant for the mucopolysaccharidoses

    Lum, S. H., Jones, S. A., Ghosh, A., Bigger, B. W. & Wynn, R. F., 24 Feb 2016, In: Expert Opinion on Orphan Drugs. 4, 4, p. 379-393 15 p.

    Research output: Contribution to journalReview articlepeer-review

  77. Published

    Enzyme replacement therapy prior to haematopoietic stem cell transplantation in Mucopolysaccharidosis Type I: 10 year combined experience of 2 centres

    Ghosh, A., Miller, W., Orchard, P. J., Jones, S. A., Mercer, J., Church, H. J., Tylee, K., Lund, T., Bigger, B. W., Tolar, J. & Wynn, R. F., 1 Mar 2016, In: Molecular genetics and metabolism. 117, 3, p. 373-377 5 p.

    Research output: Contribution to journalArticlepeer-review

  78. 2017
  79. Published

    Mucopolysaccharidosis type IIIA storage substrate drives an innate immune neuro-inflammatory response

    Bigger, B., Boutin, H., Wilkinson, F., Holley, R., Brough, D., Pinteaux, E. & Parker, H., 2017, p. S27-S28.

    Research output: Contribution to conferenceAbstractpeer-review

  80. Published

    Recommendations on clinical trial design for treatment of Mucopolysaccharidosis Type III

    Ghosh, A., Shapiro, E., Rust, S., Delaney, K., Parker, S., Shaywitz, A. J., Morte, A., Bubb, G., Cleary, M., Bo, T., Lavery, C., Bigger, B. & Jones, S., 2017, In: Orphanet Journal of Rare Diseases.

    Research output: Contribution to journalArticlepeer-review

  81. Published

    Identification of Age-Dependent Motor and Neuropsychological Behavioural Abnormalities in a Mouse Model of Mucopolysaccharidosis Type II

    Gleitz, H., O'Leary, C., Holley, R. & Bigger, B., 16 Feb 2017, In: PLoS ONE.

    Research output: Contribution to journalArticlepeer-review

  82. Published

    Sterols and oxysterols in plasma from Smith-Lemli-Opitz syndrome patients

    Griffiths, W. J., Abdel-Khalik, J., Crick, P. J., Ogundare, M., Shackleton, C. H., Tuschl, K., Kwok, M. K., Bigger, B. W., Morris, A. A., Honda, A., Xu, L., Porter, N. A., Björkhem, I., Clayton, P. T. & Wang, Y., 1 May 2017, In: Journal of Steroid Biochemistry and Molecular Biology.

    Research output: Contribution to journalArticlepeer-review

  83. 2018
  84. Published

    Anatomical changes and pathophysiology of the brain in mucopolysaccharidosis disorders

    Bigger, B. W., Begley, D. J., Virgintino, D. & Pshezhetsky, A. V., 2018, In: Molecular genetics and metabolism.

    Research output: Contribution to journalArticlepeer-review

  85. Published

    Delivering Hematopoietic Stem Cell Gene Therapy Treatments for Neurological Lysosomal Diseases.

    Holley, R., Wood, S. & Bigger, B., 2018, In: ACS Chemical Neuroscience.

    Research output: Contribution to journalReview articlepeer-review

  86. Published

    Identification Of Unusual Oxysterols And Bile acids With 7-Oxo Or 3β,5α,6β-Trihydroxy Functions In Human Plasma By Charge-Tagging Mass Spectrometry With Multistage Fragmentation

    Griffiths, W. J., Gilmore, I., Yutuc, E., Abdel-Khalik, J., Crick, P. J., Hearn, T., Dickson, A., Bigger, B. W., Goenka, A., Ghosh, A., Jones, S. A. & Wang, Y., 2018, In: Journal of Lipid Research. 59

    Research output: Contribution to journalArticlepeer-review

  87. Published

    The role of innate immunity in mucopolysaccharide diseases

    Parker, H. & Bigger, B. W., 2018, In: Journal of neurochemistry.

    Research output: Contribution to journalArticlepeer-review

  88. Published

    Macrophage enzyme and reduced inflammation drive brain correction of mucopolysaccharidosis IIIB by stem cell gene therapy

    Holley, R. J., Ellison, S. M., Fil, D., O'Leary, C., McDermott, J., Senthivel, N., Langford-Smith, A. W. W., Wilkinson, F. L., D'Souza, Z., Parker, H., Liao, A., Rowlston, S., Gleitz, H. F. E., Kan, S. H., Dickson, P. I. & Bigger, B. W., 1 Jan 2018, In: Brain. 141, 1, p. 99-116 18 p.

    Research output: Contribution to journalArticlepeer-review

  89. Published

    Interleukin-1 plays a central role in behaviour abnormalities in mucopolysaccharidosis type III (MPS HI)

    Bigger, B. W., Holley, R., Ellison, S., O'Leary, C., Pinteaux, E., Boutin, H. & Parker, H., Feb 2018, In: Molecular genetics and metabolism. 123, 2, p. S24-S25

    Research output: Contribution to journalMeeting Abstractpeer-review

  90. Published

    High content screening of patient-derived cell lines highlights the potential of non-standard chemotherapeutic agents for the treatment of glioblastoma

    Yu, K. K. H., Taylor, J. T., Pathmanaban, O. N., Youshani, A. S., Beyit, D., Dutko-Gwozdz, J., Benson, R., Griffiths, G., Peers, I., Cueppens, P., Telfer, B. A., Williams, K. J., McBain, C., Kamaly-Asl, I. D. & Bigger, B. W., 2 Mar 2018, In: PLoS ONE. 13, 3, e0193694.

    Research output: Contribution to journalArticlepeer-review

  91. Published

    A novel adeno-associated virus capsid with enhanced neurotropism corrects a lysosomal transmembrane enzyme deficiency

    Tordo, J., O'Leary, C., Antunes, A. S. L. M., Palomar, N., Aldrin-Kirk, P., Basche, M., Bennett, A., D'Souza, Z., Gleitz, H., Godwin, A., Holley, R., Parker, H., Liao, A., Rouse, P., Youshani, A., Dridi, L., Martins, C., Levade, T., Stacey, K., Davis, D. M. & 11 others, Dyer, A., Clement, N., Bjorklund, T., Ali, R. R., Agbandje-McKenna, M., Rahim, A., Pshezhetsky, A. V., Waddington, S. N., Linden, R. M., Bigger, B. & Henckaerts, E., 16 May 2018, In: Brain. 141, 7, p. 2014-2031 18 p.

    Research output: Contribution to journalArticlepeer-review

  92. Published

    Brain‐targeted stem cell gene therapy corrects mucopolysaccharidosis type II via multiple mechanisms

    Gleitz, H. F., Liao, A. Y., Cook, J. R., Rowlston, S. F., Forte, G. M., D'souza, Z., O'leary, C., Holley, R. J. & Bigger, B. W., 1 Jul 2018, In: EMBO Molecular Medicine . 10, 7, p. e8730

    Research output: Contribution to journalArticlepeer-review

  93. Published

    Substrate accumulation and extracellular matrix remodelling promote persistent upper airway disease in mucopolysaccharidosis patients on enzyme replacement therapy

    Pal, A. R., Mercer, J., Jones, S. A., Bruce, I. A., Bigger, B. W. & Dardis, A. (ed.), 18 Sep 2018, In: PLoS ONE. 13, 9, p. e0203216

    Research output: Contribution to journalArticlepeer-review

  94. 2019
  95. Published

    Metabolism of Non-Enzymatically Derived Oxysterols: Clues from sterol metabolic disorders

    Griffiths, W. J., Yutuc, E., Abdel-Khalik, J., Crick, P. J., Hearn, T., Dickson, A., Bigger, B. W., Hoi-Yee Wu, T., Goenka, A., Ghosh, A., Jones, S. A., Covey, D. F., Ory, D. S. & Wang, Y., 2019, In: Free Radical Biology and Medicine.

    Research output: Contribution to journalArticlepeer-review

  96. Published

    Non-myeloablative busulfan chimeric mouse models are less pro-inflammatory than head-shielded irradiation for studying immune cell interactions in brain tumours

    Youshani, A. S., Rowlston, S., O'Leary, C., Forte, G., Parker, H., Liao, A., Telfer, B., Williams, K., Kamaly-Asl, I. D. & Bigger, B. W., 2019, In: Journal of Neuroinflammation. 16, 1, 25.

    Research output: Contribution to journalArticlepeer-review

  97. Published

    A nonmyeloablative chimeric mouse model accurately defines microglia and macrophage contribution in glioma

    Yu, K., Youshani, A. S., Wilkinson, F. L., O'Leary, C., Cook, P., Laaniste, L., Liao, A., Mosses, D., Waugh, C., Shorrock, H., Pathmanaban, O., Macdonald, A., Kamaly-Asl, I., Roncaroli, F. & Bigger, B. W., 1 Feb 2019, In: Neuropathology and Applied Neurobiology. 45, 2, p. 119-140 22 p.

    Research output: Contribution to journalArticlepeer-review

  98. Published

    Modifying inter-cistronic sequence significantly enhances IRES dependent second gene expression in bicistronic vector: Construction of optimised cassette for gene therapy of familial hypercholesterolemia

    Al-Allaf, F. A., Abduljaleel, Z., Athar, M., Taher, M. M., Khan, W., Mehmet, H., Colakogullari, M., Apostolidou, S., Bigger, B., Waddington, S., Coutelle, C., Themis, M., Al-Ahdal, M. N., Al-Mohanna, F. A., Al-Hassnan, Z. N. & Bouazzaoui, A., Mar 2019, In: Non-coding RNA research. 4, 1, p. 1-14 14 p.

    Research output: Contribution to journalArticlepeer-review

  99. Published

    Pre-clinical Safety and Efficacy of Lentiviral Vector-Mediated Ex Vivo Stem Cell Gene Therapy for the Treatment of Mucopolysaccharidosis IIIA

    Ellison, S. M., Liao, A., Wood, S., Taylor, J., Youshani, A. S., Rowlston, S., Parker, H., Armant, M., Biffi, A., Chan, L., Farzaneh, F., Wynn, R., Jones, S. A., Heal, P., Gaspar, H. B. & Bigger, B. W., 14 Jun 2019, In: Molecular Therapy - Methods and Clinical Development. 13, p. 399-413 15 p.

    Research output: Contribution to journalArticlepeer-review

  100. Published

    Strategies for the Induction of Immune Tolerance to Enzyme Replacement Therapy in Mucopolysaccharidosis Type I

    Ghosh, A., Liao, A., O'Leary, C., Mercer, J., Tylee, K., Goenka, A., Holley, R., Jones, S. A. & Bigger, B. W., 14 Jun 2019, In: Molecular Therapy - Methods and Clinical Development. 13, p. 321-333 13 p.

    Research output: Contribution to journalArticlepeer-review

  101. Published

    An Improved Adeno-Associated Virus Vector for Neurological Correction of the Mouse Model of Mucopolysaccharidosis IIIA

    Gray, A. L., O'Leary, C., Liao, A., Agúndez, L., Youshani, A. S., Gleitz, H. F., Parker, H., Taylor, J. T., Danos, O., Hocquemiller, M., Palomar, N., Linden, R. M., Henckaerts, E., Holley, R. J. & Bigger, B. W., 17 Sep 2019, In: Human Gene Therapy. 30, 9, p. 1052-1066 15 p.

    Research output: Contribution to journalArticlepeer-review

  102. Published

    Hampering brain tumor proliferation and migration using peptide nanofiber:siPLK1/MMP2 complexes

    Mazza, M., Ahmad, H., Hadjidemetriou, M., Agliardi, G., Pathmanaban, O. N., King, A. T., Bigger, B. W., Vranic, S. & Kostarelos, K., Dec 2019, In: nanomedicine. 14, 24, p. 3127-3142 16 p.

    Research output: Contribution to journalArticlepeer-review

  103. Published

    Post-transplant laronidase augmentation for children with Hurler syndrome: biochemical outcomes

    Lund, T. C., Miller, W. P., Liao, A. Y., Tolar, J., Shanley, R., Pasquali, M., Sando, N., Bigger, B. W., Polgreen, L. E. & Orchard, P. J., 1 Dec 2019, In: Scientific Reports. 9, 1, 14105.

    Research output: Contribution to journalArticlepeer-review

  104. 2020
  105. Published

    Haematopoietic stem cell gene therapy with IL-1Ra rescues cognitive loss in mucopolysaccharidosis IIIA

    Parker, H., Ellison, S. M., Holley, R. J., O'Leary, C., Liao, A., Asadi, J., Glover, E., Ghosh, A., Jones, S., Wilkinson, F. L., Brough, D., Pinteaux, E., Boutin, H. & Bigger, B. W., 6 Mar 2020, In: EMBO Molecular Medicine. 12, 3, e11185.

    Research output: Contribution to journalArticlepeer-review

  106. Published

    Actinomycin D Downregulates Sox2 and Improves Survival in Preclinical Models of Recurrent Glioblastoma

    Taylor, J. T., Ellison, S., Pandele, A., Wood, S., Nathan, E., Forte, G., Parker, H., Zindy, E., Elvin, M., Dickson, A., Williams, K. J., Karabatsou, K., McCabe, M., McBain, C. & Bigger, B. W., 30 Mar 2020, In: Neuro-Oncology. 22, 9, p. 1289-1301 13 p.

    Research output: Contribution to journalArticlepeer-review

  107. Published

    Targeting the MAPK7/MMP9 axis for metastasis in primary bone cancer

    Green, D., Eyre, H., Singh, A., Taylor, J., Chu, J., Jeys, L., Sumathi, V. P., Coonar, A., Rassl, D., Babur, M., Forster, D., Alzabin, S., Ponthan, F., McMahon, A., Bigger, B., Reekie, T., Kassiou, M., Williams, K., Dalmay, T., Fraser, W. D. & 1 others, Finegan, K., 13 Jul 2020, In: Oncogene. 39, p. 5553–5569

    Research output: Contribution to journalArticlepeer-review

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