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

Por Gleitz, Hélène F. E., O’Leary, Claire, Holley, Rebecca J., Bigger, Brian W.
Publicado no PLoS One (2017)

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

Por Wilkinson, Fiona L, Sergijenko, Ana, Langford-Smith, Kia J, Malinowska, Marcela, Wynn, Rob F, Bigger, Brian W
Publicado em 2013

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

Por Cross, Elaine M, Grant, Sheena, Jones, Simon, Bigger, Brian W, Wraith, James E, Mahon, Louise V, Lomax, Michelle, Hare, Dougal J
Publicado no J Neurodev Disord (2014)

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

Por Langford-Smith, Alex, Langford-Smith, Kia J., Jones, Simon A., Wynn, Robert F., Wraith, J. E., Wilkinson, Fiona L., Bigger, Brian W.
Publicado em 2011

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

Por Langford-Smith, Kia J., Sandiford, Zara, Langford-Smith, Alex, Wilkinson, Fiona L., Jones, Simon A., Wraith, J. Ed, Wynn, Robert F., Bigger, Brian W.
Publicado em 2013

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

Por 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.
Publicado em 2010

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

Por 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.
Publicado em 2011

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

Por 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.
Publicado no Mol Genet Metab (2016)

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

Por 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.
Publicado em 2012

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

Por 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.
Publicado no J Biol Chem (2014)

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

Por 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
Publicado no Orphanet J Rare Dis (2015)

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

Por 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.
Publicado em 2010

Hematopoietic Stem Cell and Gene Therapy Corrects Primary Neuropathology and Behavior in Mucopolysaccharidosis IIIA Mice

Por Langford-Smith, Alexander, Wilkinson, Fiona L, Langford-Smith, Kia J, Holley, Rebecca J, Sergijenko, Ana, Howe, Steven J, Bennett, William R, Jones, Simon A, Wraith, JE, Merry, Catherine LR, Wynn, Robert F, Bigger, Brian W
Publicado em 2012

Neuropathology in Mouse Models of Mucopolysaccharidosis Type I, IIIA and IIIB

Por Wilkinson, Fiona L., Holley, Rebecca J., Langford-Smith, Kia J., Badrinath, Soumya, Liao, Aiyin, Langford-Smith, Alex, Cooper, Jonathan D., Jones, Simon A., Wraith, J. Ed, Wynn, Rob F., Merry, Catherine L. R., Bigger, Brian W.
Publicado em 2012

Myeloid/Microglial Driven Autologous Hematopoietic Stem Cell Gene Therapy Corrects a Neuronopathic Lysosomal Disease

Por Sergijenko, Ana, Langford-Smith, Alexander, Liao, Ai Y, Pickford, Claire E, McDermott, John, Nowinski, Gabriel, Langford-Smith, Kia J, Merry, Catherine LR, Jones, Simon A, Wraith, J Edmond, Wynn, Robert F, Wilkinson, Fiona L, Bigger, Brian W
Publicado em 2013

Sterols and Oxysterols in Plasma from Smith-Lemi-Opitz Syndrome Patients

Por Griffiths, William J., Abdel-Khalik, Jonas, Crick, Peter J., Ogundare, Michael, Shackleton, Cedric H., Tuschl, Karin, Kwok, Mei Kwun, Bigger, Brian W., Morris, Andrew A., Honda, Akira, Xu, Libin, Porter, Ned A., Björkhem, Ingemar, Clayton, Peter T., Wang, Yuqin
Publicado no J Steroid Biochem Mol Biol (2016)

Neuroinflammation, mitochondrial defects and neurodegeneration in mucopolysaccharidosis III type C mouse model

Por Martins, Carla, Hůlková, Helena, Dridi, Larbi, Dormoy-Raclet, Virginie, Grigoryeva, Lubov, Choi, Yoo, Langford-Smith, Alexander, Wilkinson, Fiona L., Ohmi, Kazuhiro, DiCristo, Graziella, Hamel, Edith, Ausseil, Jerôme, Cheillan, David, Moreau, Alain, Svobodová, Eva, Hájková, Zuzana, Tesařová, Markéta, Hansíková, Hana, Bigger, Brian W., Hrebícek, Martin, Pshezhetsky, Alexey V.
Publicado no Brain (2015)