Prof Brian Bigger PhD

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 journal › Article › peer-review

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 journal › Article › peer-review

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 journal › Article › peer-review

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 journal › Article › peer-review

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 journal › Article › peer-review

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 journal › Article › peer-review

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 journal › Article › peer-review

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 journal › Article › peer-review

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 journal › Article › peer-review

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 journal › Article › peer-review