Prof Brian Bigger PhD

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 journal › Article › peer-review

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 journal › Article › peer-review

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 journal › Article › peer-review

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 journal › Article › peer-review

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 journal › Article › peer-review

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 journal › Article › peer-review

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 journal › Article › peer-review

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 journal › Article › peer-review

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 journal › Article › peer-review

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 journal › Article › peer-review