Prof Brian Bigger PhD

Targeting the MAPK7/MMP9 axis for metastasis in primary bone cancer

Green, D., Eyre, H., Singh, A., Taylor, J., Chu, J., Jeys, L., Sumathi, V. P., Coonar, A., Rassl, D., Babur, M., Forster, D., Alzabin, S., Ponthan, F., McMahon, A., Bigger, B., Reekie, T., Kassiou, M., Williams, K., Dalmay, T., Fraser, W. D. & 1 others, Finegan, K., 13 Jul 2020, In: Oncogene. 39, p. 5553–5569

Research output: Contribution to journal › Article › peer-review

Actinomycin D Downregulates Sox2 and Improves Survival in Preclinical Models of Recurrent Glioblastoma

Taylor, J. T., Ellison, S., Pandele, A., Wood, S., Nathan, E., Forte, G., Parker, H., Zindy, E., Elvin, M., Dickson, A., Williams, K. J., Karabatsou, K., McCabe, M., McBain, C. & Bigger, B. W., 30 Mar 2020, In: Neuro-Oncology.

Research output: Contribution to journal › Article › peer-review

Haematopoietic stem cell gene therapy with IL-1Ra rescues cognitive loss in mucopolysaccharidosis IIIA

Parker, H., Ellison, S. M., Holley, R. J., O'Leary, C., Liao, A., Asadi, J., Glover, E., Ghosh, A., Jones, S., Wilkinson, F. L., Brough, D., Pinteaux, E., Boutin, H. & Bigger, B. W., 6 Mar 2020, In: EMBO Molecular Medicine. 12, 3, e11185.

Research output: Contribution to journal › Article › peer-review

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

Lund, T. C., Miller, W. P., Liao, A. Y., Tolar, J., Shanley, R., Pasquali, M., Sando, N., Bigger, B. W., Polgreen, L. E. & Orchard, P. J., 1 Dec 2019, In: Scientific Reports. 9, 1, 14105.

Research output: Contribution to journal › Article › peer-review

Hampering brain tumor proliferation and migration using peptide nanofiber:siPLK1/MMP2 complexes

Mazza, M., Ahmad, H., Hadjidemetriou, M., Agliardi, G., Pathmanaban, O. N., King, A. T., Bigger, B. W., Vranic, S. & Kostarelos, K., Dec 2019, In: nanomedicine. 14, 24, p. 3127-3142 16 p.

Research output: Contribution to journal › Article › peer-review

An Improved Adeno-Associated Virus Vector for Neurological Correction of the Mouse Model of Mucopolysaccharidosis IIIA

Gray, A. L., O'Leary, C., Liao, A., Agúndez, L., Youshani, A. S., Gleitz, H. F., Parker, H., Taylor, J. T., Danos, O., Hocquemiller, M., Palomar, N., Linden, R. M., Henckaerts, E., Holley, R. J. & Bigger, B. W., 17 Sep 2019, In: Human Gene Therapy. 30, 9, p. 1052-1066 15 p.

Research output: Contribution to journal › Article › peer-review

Pre-clinical Safety and Efficacy of Lentiviral Vector-Mediated Ex Vivo Stem Cell Gene Therapy for the Treatment of Mucopolysaccharidosis IIIA

Ellison, S. M., Liao, A., Wood, S., Taylor, J., Youshani, A. S., Rowlston, S., Parker, H., Armant, M., Biffi, A., Chan, L., Farzaneh, F., Wynn, R., Jones, S. A., Heal, P., Gaspar, H. B. & Bigger, B. W., 14 Jun 2019, In: Molecular Therapy - Methods and Clinical Development. 13, p. 399-413 15 p.

Research output: Contribution to journal › Article › peer-review

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

Ghosh, A., Liao, A., O'Leary, C., Mercer, J., Tylee, K., Goenka, A., Holley, R., Jones, S. A. & Bigger, B. W., 14 Jun 2019, In: Molecular Therapy - Methods and Clinical Development. 13, p. 321-333 13 p.

Research output: Contribution to journal › Article › peer-review

Modifying inter-cistronic sequence significantly enhances IRES dependent second gene expression in bicistronic vector: Construction of optimised cassette for gene therapy of familial hypercholesterolemia

Al-Allaf, F. A., Abduljaleel, Z., Athar, M., Taher, M. M., Khan, W., Mehmet, H., Colakogullari, M., Apostolidou, S., Bigger, B., Waddington, S., Coutelle, C., Themis, M., Al-Ahdal, M. N., Al-Mohanna, F. A., Al-Hassnan, Z. N. & Bouazzaoui, A., Mar 2019, In: Non-coding RNA research. 4, 1, p. 1-14 14 p.

Research output: Contribution to journal › Article › peer-review

A nonmyeloablative chimeric mouse model accurately defines microglia and macrophage contribution in glioma

Yu, K., Youshani, A. S., Wilkinson, F. L., O'Leary, C., Cook, P., Laaniste, L., Liao, A., Mosses, D., Waugh, C., Shorrock, H., Pathmanaban, O., Macdonald, A., Kamaly-Asl, I., Roncaroli, F. & Bigger, B. W., 1 Feb 2019, In: Neuropathology and Applied Neurobiology. 45, 2, p. 119-140 22 p.

Research output: Contribution to journal › Article › peer-review

Metabolism of Non-Enzymatically Derived Oxysterols: Clues from sterol metabolic disorders

Griffiths, W. J., Yutuc, E., Abdel-Khalik, J., Crick, P. J., Hearn, T., Dickson, A., Bigger, B. W., Hoi-Yee Wu, T., Goenka, A., Ghosh, A., Jones, S. A., Covey, D. F., Ory, D. S. & Wang, Y., 2019, In: Free Radical Biology and Medicine.

Research output: Contribution to journal › Article › peer-review

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

Youshani, A. S., Rowlston, S., O'Leary, C., Forte, G., Parker, H., Liao, A., Telfer, B., Williams, K., Kamaly-Asl, I. D. & Bigger, B. W., 2019, In: Journal of Neuroinflammation. 16, 1, 25.

Research output: Contribution to journal › Article › peer-review

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

Pal, A. R., Mercer, J., Jones, S. A., Bruce, I. A., Bigger, B. W. & Dardis, A. (ed.), 18 Sep 2018, In: PLoS ONE. 13, 9, p. e0203216

Research output: Contribution to journal › Article › peer-review

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

Gleitz, H. F., Liao, A. Y., Cook, J. R., Rowlston, S. F., Forte, G. M., D'souza, Z., O'leary, C., Holley, R. J. & Bigger, B. W., 1 Jul 2018, In: EMBO Molecular Medicine . 10, 7, p. e8730

Research output: Contribution to journal › Article › peer-review

A novel adeno-associated virus capsid with enhanced neurotropism corrects a lysosomal transmembrane enzyme deficiency

Tordo, J., O'Leary, C., Antunes, A. S. L. M., Palomar, N., Aldrin-Kirk, P., Basche, M., Bennett, A., D'Souza, Z., Gleitz, H., Godwin, A., Holley, R., Parker, H., Liao, A., Rouse, P., Youshani, A., Dridi, L., Martins, C., Levade, T., Stacey, K., Davis, D. M. & 11 others, Dyer, A., Clement, N., Bjorklund, T., Ali, R. R., Agbandje-McKenna, M., Rahim, A., Pshezhetsky, A. V., Waddington, S. N., Linden, R. M., Bigger, B. & Henckaerts, E., 16 May 2018, In: Brain. 141, 7, p. 2014-2031 18 p.

Research output: Contribution to journal › Article › peer-review

High content screening of patient-derived cell lines highlights the potential of non-standard chemotherapeutic agents for the treatment of glioblastoma

Yu, K. K. H., Taylor, J. T., Pathmanaban, O. N., Youshani, A. S., Beyit, D., Dutko-Gwozdz, J., Benson, R., Griffiths, G., Peers, I., Cueppens, P., Telfer, B. A., Williams, K. J., McBain, C., Kamaly-Asl, I. D. & Bigger, B. W., 2 Mar 2018, In: PLoS ONE. 13, 3, e0193694.

Research output: Contribution to journal › Article › peer-review

Interleukin-1 plays a central role in behaviour abnormalities in mucopolysaccharidosis type III (MPS HI)

Bigger, B. W., Holley, R., Ellison, S., O'Leary, C., Pinteaux, E., Boutin, H. & Parker, H., Feb 2018, In: Molecular genetics and metabolism. 123, 2, p. S24-S25

Research output: Contribution to journal › Meeting Abstract › peer-review

Macrophage enzyme and reduced inflammation drive brain correction of mucopolysaccharidosis IIIB by stem cell gene therapy

Holley, R. J., Ellison, S. M., Fil, D., O'Leary, C., McDermott, J., Senthivel, N., Langford-Smith, A. W. W., Wilkinson, F. L., D'Souza, Z., Parker, H., Liao, A., Rowlston, S., Gleitz, H. F. E., Kan, S. H., Dickson, P. I. & Bigger, B. W., 1 Jan 2018, In: Brain. 141, 1, p. 99-116 18 p.

Research output: Contribution to journal › Article › peer-review

Anatomical changes and pathophysiology of the brain in mucopolysaccharidosis disorders

Bigger, B. W., Begley, D. J., Virgintino, D. & Pshezhetsky, A. V., 2018, In: Molecular genetics and metabolism.

Research output: Contribution to journal › Article › peer-review

Delivering Hematopoietic Stem Cell Gene Therapy Treatments for Neurological Lysosomal Diseases.

Holley, R., Wood, S. & Bigger, B., 2018, In: ACS Chemical Neuroscience.

Research output: Contribution to journal › Review article › peer-review

Identification Of Unusual Oxysterols And Bile acids With 7-Oxo Or 3β,5α,6β-Trihydroxy Functions In Human Plasma By Charge-Tagging Mass Spectrometry With Multistage Fragmentation

Griffiths, W. J., Gilmore, I., Yutuc, E., Abdel-Khalik, J., Crick, P. J., Hearn, T., Dickson, A., Bigger, B. W., Goenka, A., Ghosh, A., Jones, S. A. & Wang, Y., 2018, In: Journal of Lipid Research. 59

Research output: Contribution to journal › Article › peer-review

The role of innate immunity in mucopolysaccharide diseases

Parker, H. & Bigger, B. W., 2018, In: Journal of neurochemistry.

Research output: Contribution to journal › Article › peer-review

Sterols and oxysterols in plasma from Smith-Lemli-Opitz syndrome patients

Griffiths, W. J., Abdel-Khalik, J., Crick, P. J., Ogundare, M., Shackleton, C. H., Tuschl, K., Kwok, M. K., Bigger, B. W., Morris, A. A., Honda, A., Xu, L., Porter, N. A., Björkhem, I., Clayton, P. T. & Wang, Y., 1 May 2017, In: Journal of Steroid Biochemistry and Molecular Biology.

Research output: Contribution to journal › Article › peer-review

Identification of Age-Dependent Motor and Neuropsychological Behavioural Abnormalities in a Mouse Model of Mucopolysaccharidosis Type II

Gleitz, H., O'Leary, C., Holley, R. & Bigger, B., 16 Feb 2017, In: PLoS ONE.

Research output: Contribution to journal › Article › peer-review

Recommendations on clinical trial design for treatment of Mucopolysaccharidosis Type III

Ghosh, A., Shapiro, E., Rust, S., Delaney, K., Parker, S., Shaywitz, A. J., Morte, A., Bubb, G., Cleary, M., Bo, T., Lavery, C., Bigger, B. & Jones, S., 2017, In: Orphanet Journal of Rare Diseases.

Research output: Contribution to journal › Article › peer-review

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

Ghosh, A., Miller, W., Orchard, P. J., Jones, S. A., Mercer, J., Church, H. J., Tylee, K., Lund, T., Bigger, B. W., Tolar, J. & Wynn, R. F., 1 Mar 2016, In: Molecular genetics and metabolism. 117, 3, p. 373-377 5 p.

Research output: Contribution to journal › Article › peer-review

Hematopoietic stem cell transplant for the mucopolysaccharidoses

Lum, S. H., Jones, S. A., Ghosh, A., Bigger, B. W. & Wynn, R. F., 24 Feb 2016, In: Expert Opinion on Orphan Drugs. 4, 4, p. 379-393 15 p.

Research output: Contribution to journal › Review article › peer-review

The impact of the immune system on the safety and efficiency of enzyme replacement therapy in lysosomal storage disorders

Broomfield, A., Jones, S. A., Hughes, S. M. & Bigger, B. W., 16 Feb 2016, In: Journal of Inherited Metabolic Disease. 39, 4, p. 499-512 14 p.

Research output: Contribution to journal › Article › peer-review

In vivo T-cell depletion using alemtuzumab in family and unrelated donor transplantation for pediatric non-malignant disease achieves engraftment with low incidence of graft vs. host disease.

Saif, M. A., Borrill, R., Bigger, B. W., Lee, H., Logan, A., Poulton, K., Hughes, S., Turner, A. J., Bonney, D. K. & Wynn, R. F., Mar 2015, In: Pediatric transplantation. 19, 2

Research output: Contribution to journal › Article › peer-review

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

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

Cross, E. M., Grant, S., Jones, S., Bigger, B. W., Wraith, J. E., Mahon, L. V., Lomax, M. & Hare, D. J., 2014, In: Journal of neurodevelopmental disorders. 6, 1

Research output: Contribution to journal › Article › peer-review

Novel approaches and mechanisms in hematopoietic stem cell gene therapy

Bigger, B. W. & Wynn, R. F., 2014, In: Discovery Medicine. 17, 94

Research output: Contribution to journal › Article › peer-review

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

Langford-Smith, K. J., Sandiford, Z., Langford-Smith, A., Wilkinson, F. L., Jones, S. A., Wraith, J. E., Wynn, R. F. & Bigger, B. W., 17 Oct 2013, In: PLoS ONE. 8, 10, e77632.

Research output: Contribution to journal › Article › peer-review

Myeloid/Microglial driven autologous hematopoietic stem cell gene therapy corrects a neuronopathic lysosomal disease

Sergijenko, A., Langford-Smith, A., Liao, A. Y., Pickford, C. E., McDermott, J., Nowinski, G., Langford-Smith, K. J., Merry, C. L., Jones, S. A., Wraith, J. E., Wynn, R. F., Wilkinson, F. L. & Bigger, B. W., Oct 2013, In: Molecular Therapy. 21, 10, p. 1938-1949 11 p.

Research output: Contribution to journal › Article › peer-review

Central and haematopoietic interleukin-1 both contribute to ischaemic brain injury in mice

Denes, A., Wilkinson, F., Bigger, B., Chu, M., Rothwell, N. J. & Allan, S. M., Jul 2013, In: DMM Disease Models and Mechanisms. 6, 4, p. 1043-1048 5 p.

Research output: Contribution to journal › Article › peer-review

Analytical strategies for characterization of oxysterol lipidomes: Liver X receptor ligands in plasma

Griffiths, W. J., Crick, P. J., Wang, Y., Ogundare, M., Tuschl, K., Morris, A. A., Bigger, B. W., Clayton, P. T. & Wang, Y., Jun 2013, In: Free Radical Biology and Medicine. 59, p. 69-84 15 p.

Research output: Contribution to journal › Article › peer-review

Busulfan conditioning enhances engraftment of hematopoietic donor-derived cells in the brain compared with irradiation

Wilkinson, F. L., Sergijenko, A., Langford-Smith, K. J., Malinowska, M., Wynn, R. F. & Bigger, B. W., Apr 2013, In: Molecular Therapy. 21, 4, p. 868-876 8 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