Prof Brian Bigger PhD

Tipping the scales in favour of mitochondrial gene therapy

Bigger, B., Collombet, J. M. & Coutelle, C., Dec 1999, In: Gene Therapy. 6, 12, p. 1909-1910 1 p.

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

Introduction of chloramphenicol resistance into the modified mouse mitochondrial genome: Cloning of unstable sequences by passage through yeast

Bigger, B., Tolmachov, O., Collombet, J. M. & Coutelle, C., 15 Jan 2000, In: Analytical Biochemistry. 277, 2, p. 236-242 6 p.

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

Gentherapie der zystischen fibrose: Eine analyse der bisherigen klinischen studien

Coutelle, C. & Bigger, B., 2001, In: Internist. 42, 10, p. 1346-1356 10 p.

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

Perspectives on gene therapy for cystic fibrosis airway disease

Bigger, B. & Coutelle, C., 2001, In: BioDrugs. 15, 9, p. 615-634 19 p.

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

An araC-controlled Bacterial cre Expression System to Produce DNA Minicircle Vectors for Nuclear and Mitochondrial Gene Therapy

Bigger, B. W., Tolmachov, O., Collombet, J. M., Fragkos, M., Palaszewski, I. & Coutelle, C., 22 Jun 2001, In: Journal of Biological Chemistry. 276, 25, p. 23018-23027 9 p.

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

Long-term transgene expression by administration of a lentivirus-based vector to the fetal circulation of immuno-competent mice

Waddington, S. N., Mitrophanous, K. A., Ellard, F. M., Buckley, S. M. K., Nivsarkar, M., Lawrence, L., Cook, H. T., Al-Allaf, F., Bigger, B., Kingsman, S. M., Coutelle, C. & Themis, M., Aug 2003, In: Gene Therapy. 10, 15, p. 1234-1240 6 p.

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

Hepatic stem cells: From inside and outside the liver?

Alison, M. R., Vig, P., Russo, F., Bigger, B. W., Amofah, E., Themis, M. & Forbes, S., Feb 2004, In: Cell Proliferation. 37, 1, p. 1-21 20 p.

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

Development of a Self-assembling Nuclear Targeting Vector System Based on the Tetracycline Repressor Protein

Vaysse, L., Harbottle, R., Bigger, B., Bergau, A., Tolmachov, O. & Coutelle, C., 13 Feb 2004, In: Journal of Biological Chemistry. 279, 7, p. 5555-5564 9 p.

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

Highly efficient EIAV-mediated in utero gene transfer and expression in the major muscle groups affected by Duchenne muscular dystrophy

Bigger, B., Gregory, L. G., Waddington, S. N., Holder, M. V., Mitrophanous, K. A., Buckley, S. M. K., Mosley, K. L., Bigger, B. W., Ellard, F. M., Walmsley, L. E., Lawrence, L., Al-Allaf, F., Kingsman, S., Coutelle, C. & Themis, M., Jul 2004, In: Gene Therapy. 11, 14, p. 1117-1125 8 p.

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

Permanent phenotypic correction of hemophilia B in immunocompetent mice by prenatal gene therapy

Waddington, S. N., Nivsarkar, M. S., Mistry, A. R., Buckley, S. M. K., Kemball-Cook, G., Mosley, K. L., Mitrophanous, K., Radcliffe, P., Holder, M. V., Brittan, M., Georgiadis, A., Al-Allaf, F., Bigger, B. W., Gregory, L. G., Terence Cook, H., Ali, R. R., Thrasher, A., Tuddenham, E. G. D., Themis, M. & Coutelle, C., 1 Nov 2004, In: Blood. 104, 9, p. 2714-2721 7 p.

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

Comparison of HIV- and EIAV-based vectors on their efficiency in transducing murine and human hematopoietic repopulating cells

Siapati, E. K., Bigger, B. W., Miskin, J., Chipchase, D., Parsley, K. L., Mitrophanous, K., Themis, M., Thrasher, A. J. & Bonnet, D., Sep 2005, In: Molecular Therapy. 12, 3, p. 537-546 9 p.

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

Oncogenesis following delivery of a nonprimate lentiviral gene therapy vector to fetal and neonatal mice

Themis, M., Waddington, S. N., Schmidt, M., von Kalle, C., Wang, Y., Al-Allaf, F., Gregory, L. G., Nivsarkar, M., Themis, M., Holder, M. V., Buckley, S. M. K., Dighe, N., Ruthe, A. T., Mistry, A., Bigger, B., Rahim, A., Nguyen, T. H., Trono, D., Thrasher, A. J. & Coutelle, C., Oct 2005, In: Molecular Therapy. 12, 4, p. 763-771 8 p.

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

Permanent partial phenotypic correction and tolerance in a mouse model of hemophilia B by stem cell gene delivery of human factor IX

Bigger, B., Siapati, E. K., Mistry, A., Waddington, S. N., Nivsarkar, M. S., Jacobs, L., Perrett, R., Holder, M. V., Ridler, C., Kemball-Cook, G., Ali, R. R., Forbes, S. J., Coutelle, C., Wright, N., Alison, M., Thrasher, A. J., Bonnet, D. & Themis, M., Jan 2006, In: Gene Therapy. 13, 2, p. 117-126 9 p.

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

RecET driven chromosomal gene targeting to generate a RecA deficient Escherichia coli strain for Cre mediated production of minicircle DNA

Tolmachov, O., Palaszewski, I., Bigger, B. & Coutelle, C., 10 Mar 2006, In: BMC Biotechnology. 6, 17.

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

The Bone Marrow Functionally Contributes to Liver Fibrosis

Russo, F. P., Alison, M. R., Bigger, B. W., Amofah, E., Florou, A., Amin, F., Bou-Gharios, G., Jeffery, R., Iredale, J. P. & Forbes, S. J., Jun 2006, In: Gastroenterology. 130, 6, p. 1807-1821 14 p.

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

Murine leukemia following irradiation conditioning for transplantation of lentivirally-modified hematopoietic stem cells

Siapati, E. K., Bigger, B. W., Kashofer, K., Themis, M., Thrasher, A. J. & Bonnet, D., Apr 2007, In: European journal of haematology. 78, 4, p. 303-313 10 p.

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

Xenogenic transfer of isolated murine mitochondria into human ρ0 cells can improve respiratory function

Katrangi, E., D'Souza, G., Boddapati, S. V., Kulawiec, M., Singh, K. K., Bigger, B. & Weissig, V., 1 Dec 2007, In: Rejuvenation Research. 10, 4, p. 561-570 9 p.

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

Improved Metabolic Correction in Patients with Lysosomal Storage Disease Treated with Hematopoietic Stem Cell Transplant Compared with Enzyme Replacement Therapy

Wynn, R. F., Wraith, J. E., Mercer, J., O'Meara, A., Tylee, K., Thornley, M., Church, H. J. & Bigger, B. W., Apr 2009, In: Journal Of Pediatrics. 154, 4, p. 609-611 2 p.

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

Cellular therapy of lysosomal storage disorders: Current status and future prospects

Wynn, R. F., Stubbs, M., Ozyilmaz, N., Wraith, J. E. & Bigger, B., Aug 2009, In: Current Pediatric Reviews. 5, 3, p. 147-159 12 p.

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

Genistein reduces lysosomal storage in peripheral tissues of mucopolysaccharide IIIB mice

Malinowska, M., Wilkinson, F. L., Bennett, W., Langford-Smith, K. J., O'Leary, H. A., Jakobkiewicz-Banecka, J., Wynn, R., Wraith, J. E., Wegrzyn, G. & Bigger, B. W., Nov 2009, In: Molecular genetics and metabolism. 98, 3, p. 235-242 7 p.

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

Genistein improves neuropathology and corrects behaviour in a mouse model of neurodegenerative metabolic disease

Malinowska, M., Wilkinson, F. L., Langford-Smith, K. J., Langford-Smith, A., Brown, J. R., Crawford, B. E., Vanier, M. T., Grynkiewicz, G., Wynn, R. F., Ed Wraith, J., Wegrzyn, G. & Bigger, B. W., 2010, In: PLoS ONE. 5, 12, e14192.

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

Successful allogeneic bone marrow transplant for Niemann-Pick disease type C2 is likely to be associated with a severe 'graft versus substrate' effect

Bonney, D. K., O'Meara, A., Shabani, A., Imrie, J., Bigger, B. W., Jones, S., Wraith, J. E. & Wynn, R. F., 2010, In: Journal of Inherited Metabolic Disease. 33, 3, p. S171-S173

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

Immune activation or immunomodulation in the brains of MPS IIIB mice? Commentary on "innate and adaptive immune activation in the brain of MPS IIIB mouse model"

Archer, L. D., Langford, K., Bigger, B. & Fildes, J. E., 1 Feb 2010, In: Journal of Neuroscience Research. 88, 2, p. 233

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

Evaluation of heparin cofactor II-thrombin complex as a biomarker on blood spots from mucopolysaccharidosis I, IIIA and IIIB mice

Langford-Smith, K., Arasaradnam, M., Wraith, J. E., Wynn, R. & Bigger, B. W., Mar 2010, In: Molecular genetics and metabolism. 99, 3, p. 269-274 5 p.

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

Circadian rhythm and suprachiasmatic nucleus alterations in the mouse model of mucopolysaccharidosis IIIB

Canal, M. M., Wilkinson, F. L., Cooper, J. D., Ed Wraith, J., Wynn, R. & Bigger, B. W., 19 Jun 2010, In: Behavioural brain research. 209, 2, p. 212-220 8 p.

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

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

Langford-Smith, K. J., Mercer, J., Petty, J., Tylee, K., Church, H., Roberts, J., Moss, G., Jones, S., Wynn, R., Wraith, J. E. & Bigger, B. W., Apr 2011, In: Journal of Inherited Metabolic Disease. 34, 2, p. 499-508 9 p.

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

Chronic norovirus infection in pediatric hematopoietic stem cell transplant recipients: A cause of prolonged intestinal failure requiring intensive nutritional support

Saif, M. A., Bonney, D. K., Bigger, B., Forsythe, L., Williams, N., Page, J., Babiker, Z. O., Guiver, M., Turner, A. J., Hughes, S. & Wynn, R. F., Aug 2011, In: Pediatric transplantation. 15, 5, p. 505-509 4 p.

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

Hyperactive behaviour in the mouse model of mucopolysaccharidosis iiib in the open field and home cage environments

Langford-Smith, A., Malinowska, M., Langford-Smith, K. J., Wegrzyn, G., Jones, S., Wynn, R., Wraith, J. E., Wilkinson, F. L. & Bigger, B. W., Aug 2011, In: Genes, Brain and Behavior. 10, 6, p. 673-682 9 p.

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

Female mucopolysaccharidosis IIIA mice exhibit hyperactivity and a reduced sense of danger in the open field test

Langford-Smith, A., Langford-Smith, K. J., Jones, S. A., Wynn, R. F., Wraith, J. E., Wilkinson, F. L. & Bigger, B. W., 18 Oct 2011, In: PLoS ONE. 6, 10, e25717.

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

Mucopolysaccharidosis type I, unique structure of accumulated heparan sulfate and increased N-sulfotransferase activity in mice lacking α-L-iduronidase

Holley, R. J., Deligny, A., Wei, W., Watson, H. A., Niñonuevo, M. R., Dagälv, A., Leary, J. A., Bigger, B. W., Kjellén, L. & Merry, C. L. R., 28 Oct 2011, In: Journal of Biological Chemistry. 286, 43, p. 37515-37524 9 p.

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

Trial and error: How the unclonable human mitochondrial genome was cloned in yeast

Bigger, B. W., Liao, A. Y., Sergijenko, A. & Coutelle, C., Nov 2011, In: Pharmaceutical Research. 28, 11, p. 2863-2870 7 p.

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

Neuropathology in mouse models of mucopolysaccharidosis type I, IIIA and IIIB

Wilkinson, F. L., Holley, R. J., Langford-Smith, K. J., Badrinath, S., Liao, A., Langford-Smith, A., Cooper, J. D., Jones, S. A., Wraith, J. E., Wynn, R. F., Merry, C. L. R. & Bigger, B. W., 27 Apr 2012, In: P L o S One. 7, 4, e35787.

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

Hematopoietic stem cell and gene therapy corrects primary neuropathology and behavior in mucopolysaccharidosis IIIA mice

Langford-Smith, A., Wilkinson, F. L., Langford-Smith, K. J., Holley, R. J., Sergijenko, A., Howe, S. J., Bennett, W. R., Jones, S. A., Wraith, J. E., Merry, C. L. R., Wynn, R. F. & Bigger, B. W., Aug 2012, In: Molecular Therapy. 20, 8, p. 1610-1621 11 p.

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

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

Saif, M., Bigger, B., Brookes, K., Mercer, J., Tylee, K. L., Church, H. J., Bonney, D. K., Jones, S., Ed Wraith, J. & Wynn, R. F., 1 Sep 2012, In: Haematologica. 97, 9, p. 1320-1328 8 p.

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

The fetal mouse is a sensitive genotoxicity model that exposes lentiviral-associated mutagenesis resulting in liver oncogenesis

Nowrouzi, A., Cheung, W. T., Li, T., Zhang, X., Arens, A., Paruzynski, A., Waddington, S. N., Osejindu, E., Reja, S., Von Kalle, C., Wang, Y., Al-Allaf, F., Gregory, L., Themes, M., Holder, M., Dighe, N., Ruthe, A., Buckley, S. M. K., Bigger, B., Montini, E. & 7 others, Thrasher, A. J., Andrews, R., Roberts, T. P., Newbold, R. F., Coutelle, C., Schmidt, M. & Themis, M., Feb 2013, In: Molecular Therapy. 21, 2, p. 324-337 13 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

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

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

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

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

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

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

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

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

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