Prof Brian Bigger PhD

Professor of Cell and Gene Therapy

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Publications

  1. 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

  2. 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

  3. 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

  4. 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

  5. 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

  6. 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

  7. 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

  8. 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

  9. 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

  10. 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

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Research output: Contribution to journalArticlepeer-review

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Research output: Contribution to journalReview articlepeer-review