Biblio

Author Title Type [ Year(Asc)]
Filters: Author is Bigger, Brian W  [Clear All Filters]
2024
H Y Wu T, Brown HA, Church HJ, Kershaw CJ, Hutton R, Egerton C, Cooper J, Tylee K, Cohen RN, Gokhale D, et al. Improving newborn screening test performance for metachromatic leukodystrophy: Recommendation from a pre-pilot study that identified a late-infantile case for treatment. Mol Genet Metab. 2024;142(1):108349.
2023
O'Leary C, Forte G, Mitchell NL, Youshani ASaam, Dyer A, Wellby MP, Russell KN, Murray SJ, Jolinon N, Jones SA, et al. Intraparenchymal convection enhanced delivery of AAV in sheep to treat Mucopolysaccharidosis IIIC. J Transl Med. 2023;21(1):437.
Andreou T, Ishikawa-Learmonth Y, Bigger BW. Phenotypic characterisation of the Mucopolysaccharidosis Type I (MPSI) Idua-W392X mouse model reveals increased anxiety-related traits in female mice. Mol Genet Metab. 2023;139(4):107651.
Ellison S, Liao A, Gleitz HFE, Parker H, Booth L, Robinson J, Wood S, Taylor J, Holley R, Bigger BW. Sustained long-term disease correction in a murine model of MPSII following stem cell gene therapy. Mol Ther Methods Clin Dev. 2023;31:101127.
2020
Taylor JT, Ellison S, Pandele A, Wood S, Nathan E, Forte G, Parker H, Zindy E, Elvin M, Dickson A, et al. Actinomycin D Downregulates Sox2 and Improves Survival in Preclinical Models of Recurrent Glioblastoma. Neuro Oncol. 2020.
Abdel-Khalik J, Hearn T, Dickson AL, Crick PJ, Yutuc E, Austin-Muttit K, Bigger BW, Morris AA, Shackleton CH, Clayton PT, et al. Bile Acid Biosynthesis in Smith-Lemli-Opitz Syndrome Bypassing Cholesterol: Potential Importance of Pathway Intermediates. J Steroid Biochem Mol Biol. 2020:105794.
2019
Griffiths WJ, Yutuc E, Abdel-Khalik J, Crick PJ, Hearn T, Dickson A, Bigger BW, Wu THoi-Yee, Goenka A, Ghosh A, et al. Metabolism of Non-Enzymatically Derived Oxysterols: Clues from sterol metabolic disorders. Free Radic Biol Med. 2019.
2018
Bigger BW, Begley DJ, Virgintino D, Pshezhetsky AV. Anatomical changes and pathophysiology of the brain in mucopolysaccharidosis disorders. Mol Genet Metab. 2018.
Pal ARicky, Mercer J, Jones SA, Bruce IA, Bigger BW. Substrate accumulation and extracellular matrix remodelling promote persistent upper airway disease in mucopolysaccharidosis patients on enzyme replacement therapy. PLoS One. 2018;13(9):e0203216.
2013
Langford-Smith KJ, Sandiford Z, Langford-Smith A, Wilkinson FL, Jones SA, J Wraith E, Wynn RF, Bigger BW. Signal One and Two Blockade Are Both Critical for Non-Myeloablative Murine HSCT across a Major Histocompatibility Complex Barrier. PLoS One. 2013;8(10):e77632.