Prof Brian Bigger PhD

Professor of Cell and Gene Therapy

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Publications

  1. 2015
  2. 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

  3. 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

  4. 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

  5. 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

  6. 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

  7. 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

  8. 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

  9. 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

  10. 2016
  11. 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

  12. 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

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Research output: Contribution to journalArticlepeer-review

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Research output: Contribution to journalReview articlepeer-review