Výsledky vyhledávání - "J. Ed Wraith"

High dose genistein in Sanfilippo syndrome:a randomised controlled trial

Autor: Frédéric M. Vaz, Kia J. Langford-Smith, Claire O'Leary, Stewart Rust, Karen Tylee, Maria Mercè Canal, Arunabha Ghosh, Frits A. Wijburg, Simon Jones, Andy Vail, Brian W. Bigger, Helen Parker, Michelle Hepburn, Daniel Weisberg, Catherine Breen, J. Ed Wraith

Publikováno v: Ghosh, A, Rust, S, Langford-Smith, K, Weisberg, D, Canal, M, Breen, C, Hepburn, M, Tylee, K, Vaz, F M, Vail, A, Wijburg, F, O'Leary, C, Parker, H, Wraith, E, Bigger, B W & Jones, S A 2021, ' High dose genistein in Sanfilippo syndrome : a randomised controlled trial ', Journal of Inherited Metabolic Disease . https://doi.org/10.1002/jimd.12407
Journal of inherited metabolic disease, 44(5), 1248-1262. Springer Netherlands

The aim of this study was to evaluate the efficacy of high dose genistein aglycone in Sanfilippo syndrome (mucopolysaccharidosis type III). High doses of genistein aglycone have been shown to correct neuropathology and hyperactive behaviour in mice,

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::e0a0d45660f2650ae6248545f665d9a0
https://www.research.manchester.ac.uk/portal/en/publications/high-dose-genistein-in-sanfilippo-syndrome(03230acd-691f-45e2-8555-54aa6956085b).html

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Akademický článek

Signal one and two blockade are both critical for non-myeloablative murine HSCT across a major histocompatibility complex barrier.

Autor: Kia J Langford-Smith, Zara Sandiford, Alex Langford-Smith, Fiona L Wilkinson, Simon A Jones, J Ed Wraith, Robert F Wynn, Brian W Bigger

Publikováno v: PLoS ONE, Vol 8, Iss 10, p e77632 (2013)

Non-myeloablative allogeneic haematopoietic stem cell transplantation (HSCT) is rarely achievable clinically, except where donor cells have selective advantages. Murine non-myeloablative conditioning regimens have limited clinical success, partly thr

Externí odkaz: https://doaj.org/article/d4dd05eb1e8b4b4c9bd7225f5277a426

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Akademický článek

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

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

Publikováno v: PLoS ONE, Vol 7, Iss 4, p e35787 (2012)

Mucopolysaccharide diseases (MPS) are caused by deficiency of glycosaminoglycan (GAG) degrading enzymes, leading to GAG accumulation. Neurodegenerative MPS diseases exhibit cognitive decline, behavioural problems and shortened lifespan. We have chara

Externí odkaz: https://doaj.org/article/795f2465ae6c40f69f556671f049448c

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Akademický článek

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

Autor: Marcelina Malinowska, Fiona L Wilkinson, Kia J Langford-Smith, Alex Langford-Smith, Jillian R Brown, Brett E Crawford, Marie T Vanier, Grzegorz Grynkiewicz, Rob F Wynn, J Ed Wraith, Grzegorz Wegrzyn, Brian W Bigger

Publikováno v: PLoS ONE, Vol 5, Iss 12, p e14192 (2010)

BackgroundNeurodegenerative metabolic disorders such as mucopolysaccharidosis IIIB (MPSIIIB or Sanfilippo disease) accumulate undegraded substrates in the brain and are often unresponsive to enzyme replacement treatments due to the impermeability of

Externí odkaz: https://doaj.org/article/bf2a9870d0f24a90bfb8ff75c88c7804

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

Autor: Gill Moss, Jane Roberts, Simon Jones, Kia J. Langford-Smith, Brian W. Bigger, Karen Tylee, June Petty, J. Ed Wraith, Jean Mercer, Heather J. Church, Rob Wynn

Publikováno v: Journal of Inherited Metabolic Disease

Early detection of mucopolysaccharidosis (MPS) is an important factor in treatment success; therefore, good disease biomarkers are vital. We evaluate heparin cofactor II-thrombin complex (HCII-T) as a biomarker in serum and dried blood spots (DBS) of

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::c29a5441b846008694fb055b6fdffdfe
https://doi.org/10.1007/s10545-010-9254-8

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

Autor: Jonathan D. Cooper, Brian W. Bigger, Maria Mercè Canal, Fiona L. Wilkinson, J. Ed Wraith, Rob Wynn

Publikováno v: Behavioural Brain Research. 209:212-220

Mucopolysaccharidosis IIIB (MPSIIIB) is a lysosomal storage disease characterised by progressive central nervous system degeneration in patients, with death usually in the late teens. Serious behavioural problems have been reported in children at the

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::dbb96f37045c5d309ecfa2ceebc225b3
https://doi.org/10.1016/j.bbr.2010.01.045

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

Autor: Kia J. Langford-Smith, Rob Wynn, J. Ed Wraith, Malani Arasaradnam, Brian W. Bigger

Publikováno v: Molecular Genetics and Metabolism. 99:269-274

Mucopolysaccharide (MPS) diseases are lysosomal storage disorders caused by deficiencies of enzymes catabolising glycosaminoglycans (GAGs). Abnormal GAG accumulation leads to symptoms including severe progressive neurological decline, skeletal deform

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::c667519089faa6b22b1ffaa3156ef705
https://doi.org/10.1016/j.ymgme.2009.10.175

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