Search Results - "Bigger, Brian W"

Current and Future Treatment of Mucopolysaccharidosis (MPS) Type II: Is Brain-Targeted Stem Cell Gene Therapy the Solution for This Devastating Disorder? by Horgan, Claire, Jones, Simon A, Bigger, Brian W, Wynn, Robert

“…Mucopolysaccharidosis type II (Hunter Syndrome) is a rare, x-linked recessive, progressive, multi-system, lysosomal storage disease caused by the deficiency of…”
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Metabolism of Non-Enzymatically Derived Oxysterols: Clues from sterol metabolic disorders by Griffiths, William J., Yutuc, Eylan, Abdel-Khalik, Jonas, Crick, Peter J., Hearn, Thomas, Dickson, Alison, Bigger, Brian W., Hoi-Yee Wu, Teresa, Goenka, Anu, Ghosh, Arunabha, Jones, Simon A., Covey, Douglas F., Ory, Daniel S., Wang, Yuqin

“…Cholestane-3β,5α,6β-triol (3β,5α,6β-triol) is formed from cholestan-5,6-epoxide (5,6-EC) in a reaction catalysed by cholesterol epoxide hydrolase, following…”
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Delivering gene therapy for mucopolysaccharide diseases by Wood, Shaun R, Bigger, Brian W

“…Mucopolysaccharide diseases are a group of paediatric inherited lysosomal storage diseases that are caused by enzyme deficiencies, leading to a build-up of…”
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Quantitative charge-tags for sterol and oxysterol analysis by Crick, Peter J, William Bentley, T, Abdel-Khalik, Jonas, Matthews, Ian, Clayton, Peter T, Morris, Andrew A, Bigger, Brian W, Zerbinati, Chiara, Tritapepe, Luigi, Iuliano, Luigi, Wang, Yuqin, Griffiths, William J

“…Global sterol analysis is challenging owing to the extreme diversity of sterol natural products, the tendency of cholesterol to dominate in abundance over all…”
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Gene-modified neural progenitor cells for the treatment of neuropathic lysosomal storage diseases by Mandolfo, Oriana, Bigger, Brian

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

“…The pediatric lysosomal storage disorder mucopolysaccharidosis type II is caused by mutations in IDS, resulting in accumulation of heparan and dermatan…”
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Substrate accumulation and extracellular matrix remodelling promote persistent upper airway disease in mucopolysaccharidosis patients on enzyme replacement therapy by Pal, Abhijit Ricky, Mercer, Jean, Jones, Simon A, Bruce, Iain A, Bigger, Brian W

“…Mucopolysaccharide diseases are a group of lysosomal storage disorders caused by deficiencies of hydrolase enzymes, leading to pathological glycosaminoglycan…”
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Haematopoietic stem cell gene therapy with IL‐1Ra rescues cognitive loss in mucopolysaccharidosis IIIA by Parker, Helen, Ellison, Stuart M, Holley, Rebecca J, O'Leary, Claire, Liao, Aiyin, Asadi, Jalal, Glover, Emily, Ghosh, Arunabha, Jones, Simon, Wilkinson, Fiona L, Brough, David, Pinteaux, Emmanuel, Boutin, Hervé, Bigger, Brian W

“…Mucopolysaccharidosis IIIA is a neuronopathic lysosomal storage disease, characterised by heparan sulphate and other substrates accumulating in the brain…”
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Neuropathology in mouse models of mucopolysaccharidosis type I, IIIA and IIIB by 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

“…Mucopolysaccharide diseases (MPS) are caused by deficiency of glycosaminoglycan (GAG) degrading enzymes, leading to GAG accumulation. Neurodegenerative MPS…”
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The Bone Marrow Functionally Contributes to Liver Fibrosis by Russo, Francesco P., Alison, Malcolm R., Bigger, Brian W., Amofah, Eunice, Florou, Aikaterini, Amin, Farhana, Bou–Gharios, George, Jeffery, Rosemary, Iredale, John P., Forbes, Stuart J.

“…Background & Aims Bone marrow (BM) cells may transdifferentiate into or fuse with organ parenchymal cells. BM therapy shows promise in murine models of…”
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Enzyme replacement therapy and hematopoietic stem cell transplant: a new paradigm of treatment in Wolman disease by Potter, Jane E, Petts, Gemma, Ghosh, Arunabha, White, Fiona J, Kinsella, Jane L, Hughes, Stephen, Roberts, Jane, Hodgkinson, Adam, Brammeier, Kathryn, Church, Heather, Merrigan, Christine, Hughes, Joanne, Evans, Pamela, Campbell, Helen, Bonney, Denise, Newman, William G, Bigger, Brian W, Broomfield, Alexander, Jones, Simon A, Wynn, Robert F

“…Wolman disease is a rare, lysosomal storage disorder in which biallelic variants in the LIPA gene result in reduced or complete lack of lysosomal acid lipase…”
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Myeloid/Microglial Driven Autologous Hematopoietic Stem Cell Gene Therapy Corrects a Neuronopathic Lysosomal Disease by 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

“…Mucopolysaccharidosis type IIIA (MPSIIIA) is a lysosomal storage disorder caused by mutations in N-sulfoglucosamine sulfohydrolase (SGSH), resulting in heparan…”
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Systemic immune challenge exacerbates neurodegeneration in a model of neurological lysosomal disease by Mandolfo, Oriana, Parker, Helen, Aguado, Èlia, Ishikawa Learmonth, Yuko, Liao, Ai Yin, O’Leary, Claire, Ellison, Stuart, Forte, Gabriella, Taylor, Jessica, Wood, Shaun, Searle, Rachel, Holley, Rebecca J, Boutin, Hervé, Bigger, Brian W

“…Mucopolysaccharidosis type IIIA (MPS IIIA) is a rare paediatric lysosomal storage disorder, caused by the progressive accumulation of heparan sulphate,…”
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Identification of age-dependent motor and neuropsychological behavioural abnormalities in a mouse model of Mucopolysaccharidosis Type II by Gleitz, Hélène F E, O'Leary, Claire, Holley, Rebecca J, Bigger, Brian W

“…Severe mucopolysaccharidosis type II (MPS II) is a progressive lysosomal storage disease caused by mutations in the IDS gene, leading to a deficiency in the…”
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Intraparenchymal convection enhanced delivery of AAV in sheep to treat Mucopolysaccharidosis IIIC by O'Leary, Claire, Forte, Gabriella, Mitchell, Nadia L, Youshani, Amir Saam, Dyer, Adam, Wellby, Martin P, Russell, Katharina N, Murray, Samantha J, Jolinon, Nelly, Jones, Simon A, Stacey, Kevin, Davis, Daniel M, Henckaerts, Els, Palmer, David N, Kamaly-Asl, Ian, Bigger, Brian W

“…Mucopolysaccharidosis IIIC (MPSIIIC) is one of four Sanfilippo diseases sharing clinical symptoms of severe cognitive decline and shortened lifespan. The…”
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Genistein improves neuropathology and corrects behaviour in a mouse model of neurodegenerative metabolic disease by 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

“…Neurodegenerative metabolic disorders such as mucopolysaccharidosis IIIB (MPSIIIB or Sanfilippo disease) accumulate undegraded substrates in the brain and are…”
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Hematopoietic stem cell and gene therapy corrects primary neuropathology and behavior in mucopolysaccharidosis IIIA mice by 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

“…Mucopolysaccharidosis IIIA (MPS IIIA or Sanfilippo disease) is a neurodegenerative disorder caused by a deficiency in the lysosomal enzyme sulfamidase (SGSH),…”
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An Improved Adeno-Associated Virus Vector for Neurological Correction of the Mouse Model of Mucopolysaccharidosis IIIA by Gray, Anna L, O'Leary, Claire, Liao, Aiyin, Agúndez, Leticia, Youshani, Amir S, Gleitz, Hélène F, Parker, Helen, Taylor, Jessica T, Danos, Olivier, Hocquemiller, Michaël, Palomar, Nuria, Linden, R Michael, Henckaerts, Els, Holley, Rebecca J, Bigger, Brian W

“…Patients with the lysosomal storage disease mucopolysaccharidosis IIIA (MPSIIIA) lack the lysosomal enzyme N-sulfoglucosamine sulfohydrolase (SGSH), one of the…”
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Non-myeloablative busulfan chimeric mouse models are less pro-inflammatory than head-shielded irradiation for studying immune cell interactions in brain tumours by 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

“…Chimeric mouse models generated via adoptive bone marrow transfer are the foundation for immune cell tracking in neuroinflammation. Chimeras that exhibit low…”
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Analytical strategies for characterization of oxysterol lipidomes: Liver X receptor ligands in plasma by Griffiths, William J., Crick, Peter J., Wang, Yuchen, Ogundare, Michael, Tuschl, Karin, Morris, Andrew A., Bigger, Brian W., Clayton, Peter T., Wang, Yuqin

“…Bile acids, bile alcohols, and hormonal steroids represent the ultimate biologically active products of cholesterol metabolism in vertebrates. However,…”
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