Dual Purpose Vectors for Rare Neurological Diseases

Bằng Bigger, Brian W.
Xuất bản năm Mol Ther (2020)

The role of innate immunity in mucopolysaccharide diseases

Bằng Parker, Helen, Bigger, Brian W.
Xuất bản năm J Neurochem (2018)

Identification of age-dependent motor and neuropsychological behavioural abnormalities in a mouse model of Mucopolysaccharidosis Type II

Bằng Gleitz, Hélène F. E., O’Leary, Claire, Holley, Rebecca J., Bigger, Brian W.
Xuất bản năm PLoS One (2017)

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

Bằng Pal, Abhijit Ricky, Mercer, Jean, Jones, Simon A., Bruce, Iain A., Bigger, Brian W.
Xuất bản năm PLoS One (2018)

Busulfan Conditioning Enhances Engraftment of Hematopoietic Donor-derived Cells in the Brain Compared With Irradiation

Bằng Wilkinson, Fiona L, Sergijenko, Ana, Langford-Smith, Kia J, Malinowska, Marcela, Wynn, Rob F, Bigger, Brian W
Được phát hành 2013

An investigation of the middle and late behavioural phenotypes of Mucopolysaccharidosis Type-III

Bằng Cross, Elaine M, Grant, Sheena, Jones, Simon, Bigger, Brian W, Wraith, James E, Mahon, Louise V, Lomax, Michelle, Hare, Dougal J
Xuất bản năm J Neurodev Disord (2014)

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

Bằng Ghosh, Arunabha, Liao, Aiyin, O’Leary, Claire, Mercer, Jean, Tylee, Karen, Goenka, Anu, Holley, Rebecca, Jones, Simon A., Bigger, Brian W.
Xuất bản năm Mol Ther Methods Clin Dev (2019)

Female Mucopolysaccharidosis IIIA Mice Exhibit Hyperactivity and a Reduced Sense of Danger in the Open Field Test

Bằng Langford-Smith, Alex, Langford-Smith, Kia J., Jones, Simon A., Wynn, Robert F., Wraith, J. E., Wilkinson, Fiona L., Bigger, Brian W.
Được phát hành 2011

Signal One and Two Blockade Are Both Critical for Non-Myeloablative Murine HSCT across a Major Histocompatibility Complex Barrier

Bằng Langford-Smith, Kia J., Sandiford, Zara, Langford-Smith, Alex, Wilkinson, Fiona L., Jones, Simon A., Wraith, J. Ed, Wynn, Robert F., Bigger, Brian W.
Được phát hành 2013

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

Bằng Youshani, A. Saam, Rowlston, Samuel, O’Leary, Claire, Forte, Gabriella, Parker, Helen, Liao, Aiyin, Telfer, Brian, Williams, Kaye, Kamaly-Asl, Ian D., Bigger, Brian W.
Xuất bản năm J Neuroinflammation (2019)

Heparin cofactor II-thrombin complex and dermatan sulphate:chondroitin sulphate ratio are biomarkers of short- and long-term treatment effects in mucopolysaccharide diseases

Bằng Langford-Smith, Kia Jane, Mercer, Jean, Petty, June, Tylee, Karen, Church, Heather, Roberts, Jane, Moss, Gill, Jones, Simon, Wynn, Rob, Wraith, J. Ed, Bigger, Brian W.
Được phát hành 2010

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

Bằng Lund, Troy C., Miller, Weston P., Liao, Ai Yin, Tolar, Jakub, Shanley, Ryan, Pasquali, Marzia, Sando, Nicole, Bigger, Brian W., Polgreen, Lynda E., Orchard, Paul J.
Xuất bản năm Sci Rep (2019)

Mucopolysaccharidosis Type I, Unique Structure of Accumulated Heparan Sulfate and Increased N-Sulfotransferase Activity in Mice Lacking α-l-iduronidase

Bằng Holley, Rebecca J., Deligny, Audrey, Wei, Wei, Watson, H. Angharad, Niñonuevo, Milady R., Dagälv, Anders, Leary, Julie A., Bigger, Brian W., Kjellén, Lena, Merry, Catherine L. R.
Được phát hành 2011

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

Bằng Ghosh, Arunabha, Miller, Weston, Orchard, Paul J., Jones, Simon A., Mercer, Jean, Church, Heather J., Tylee, Karen, Lund, Troy, Bigger, Brian W., Tolar, Jakub, Wynn, Robert F.
Xuất bản năm Mol Genet Metab (2016)

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

Bằng Gleitz, Hélène FE, Liao, Ai Yin, Cook, James R, Rowlston, Samuel F, Forte, Gabriella MA, D'Souza, Zelpha, O'Leary, Claire, Holley, Rebecca J, Bigger, Brian W
Xuất bản năm EMBO Mol Med (2018)

Hematopoietic stem cell transplantation improves the high incidence of neutralizing allo-antibodies observed in Hurler’s syndrome after pharmacological enzyme replacement therapy

Bằng Saif, Muhammad Ameer, Bigger, Brian W., Brookes, Karen E, Mercer, Jean, Tylee, Karen L., Church, Heather J., Bonney, Denise K., Jones, Simon, Wraith, J. Ed, Wynn, Robert F.
Được phát hành 2012

Recommendations on clinical trial design for treatment of Mucopolysaccharidosis Type III

Bằng Ghosh, Arunabha, Shapiro, Elsa, Rust, Stewart, Delaney, Kathleen, Parker, Samantha, Shaywitz, Adam J, Morte, Adelaida, Bubb, Gillian, Cleary, Maureen, Bo, Tien, Lavery, Christine, Bigger, Brian W, Jones, Simon A
Xuất bản năm Orphanet J Rare Dis (2017)

Heparan Sulfate Inhibits Hematopoietic Stem and Progenitor Cell Migration and Engraftment in Mucopolysaccharidosis I

Bằng Watson, H. Angharad, Holley, Rebecca J., Langford-Smith, Kia J., Wilkinson, Fiona L., van Kuppevelt, Toin H., Wynn, Robert F., Wraith, J. Edmond, Merry, Catherine L. R., Bigger, Brian W.
Xuất bản năm J Biol Chem (2014)

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

Bằng Pal, Abhijit Ricky, Langereis, Eveline J, Saif, Muhammad A, Mercer, Jean, Church, Heather J, Tylee, Karen L, Wynn, Robert F, Wijburg, Frits A, Jones, Simon A, Bruce, Iain A, Bigger, Brian W
Xuất bản năm Orphanet J Rare Dis (2015)

Genistein Improves Neuropathology and Corrects Behaviour in a Mouse Model of Neurodegenerative Metabolic Disease

Bằng Malinowska, Marcelina, Wilkinson, Fiona L., Langford-Smith, Kia J., Langford-Smith, Alex, Brown, Jillian R., Crawford, Brett E., Vanier, Marie T., Grynkiewicz, Grzegorz, Wynn, Rob F., Wraith, J. Ed, Wegrzyn, Grzegorz, Bigger, Brian W.
Được phát hành 2010