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Yr Athro Emyr Lloyd-Evans
Tiwtor Rhanbarthol Ôl-raddedig
Ysgol y Biowyddorau
- Lloyd-EvansE@caerdydd.ac.uk
- +44 29208 74304
- Adeilad Syr Martin Evans, Ystafell Cardiff School of Biosciences, The Sir Martin Evans Building, Museum Avenue, Cardiff, CF10 3AX, Rhodfa'r Amgueddfa, Caerdydd, CF10 3AX
- Siarad Cymraeg
Trosolwyg
Trosolwg ymchwil
Mae lysosomau yn is-set o organynnau sy'n hanfodol ar gyfer swyddogaeth gellog, fel yr epitomeiddio gan y 50 o glefydau dynol sy'n cael eu hachosi gan fwtaniadau yn y genynnau sy'n amgodio lysosomal a phroteinau cysylltiedig. Mae gan fy labordy ddiddordeb yn swyddogaeth proteinau trawsmembrane lysosomal heb eu nodweddu newydd, eu rolau mewn swyddogaeth gellog arferol a'r digwyddiadau sy'n datblygu pan fydd y proteinau hyn yn dod yn gamweithredol mewn clefydau lysosomal. Mae gennym ddiddordeb arbennig mewn cymhwyso ein hymchwil ar broteinau lysosomaidd i glefydau dynol eraill, yn enwedig clefydau heintus a niwroddirywiol. Mae ymchwil lysosomal yn faes sy'n dod i'r amlwg oherwydd datblygu offer newydd sy'n gallu mesur swyddogaeth lysosomal yn y pH asidig a geir yn yr adrannau hyn. Mae fy labordy yn defnyddio'r offer hyn i ymchwilio i swyddogaeth y dosbarth enigmatig hwn o organynnau.
Cyhoeddiad
2023
- Williamson, L. J. et al. 2023. Structure of the Lysinibacillus sphaericus Tpp49Aa1 pesticidal protein elucidated from natural crystals using MHz-SFX. Proceedings of the National Academy of Sciences 120(49), article number: e2203241120. (10.1073/pnas.2203241120)
- Best, H. L., Williamson, L. J., Heath, E. A., Waller-Evans, H., Lloyd-Evans, E. and Berry, C. 2023. The role of glycoconjugates as receptors for insecticidal proteins. FEMS Microbiology Reviews 47(4) (10.1093/femsre/fuad026)
- Somogyi, A. et al. 2023. The synthetic TRPML1 agonist ML-SA1 rescues Alzheimer-related alterations of the endosomal-autophagic-lysosomal system. Journal of Cell Science 136(6), article number: jcs259875. (10.1242/jcs.259875)
- Fenn, G. D., Alenazy, D. M., Pope, S., Lloyd-Evans, E. and Waller-Evans, H. 2023. Development of a novel anthraquinone-derived fluorescent lysosomal probe. Molecular Genetics and Metabolism 138(2), article number: 107097. (10.1016/j.ymgme.2022.107097)
- Cook, S. R. and Lloyd-Evans, E. 2023. Comparing the lysosomal biotoxicity of iron oxide nanoparticles for improved lysosomal purification. Presented at: 19th Annual WORLDSymposiumTM, Orlando, Florida, 21-26 February 2023, Vol. 138. Vol. 2. Elsevier pp. 28-29., (10.1016/j.ymgme.2022.107062)
- Lloyd-Evans, E. and Evans, C. 2023. Deciphering the various mechanisms of action of miglustat in the lysosomal disorders.. Presented at: 19th Annual WORLDSymposiumTM, Orlando, Florida, 21-26 February 2023, Vol. 138. Vol. 2. Elsevier pp. 84., (10.1016/j.ymgme.2022.107213)
- Best, H. L., Badell-Grau, R. A., Shipley, K., Alshehri, A. S., Waller-Evans, H. and Lloyd-Evans, E. 2023. Disease phenotypes present in CLN7 patient fibroblasts are improved following treatment to reduce glycosphingolipid synthesis.. Presented at: 19th Annual WORLDSymposium™ 2023, Orlando, Florida, 21-26 February 2023, Vol. 138. Vol. 2. Elsevier pp. 17., (10.1016/j.ymgme.2022.107029)
- Waller-Evans, H., Francis, L., Fenn, G., Best, H. and Lloyd-Evans, E. 2023. Filipin composition and imaging modality dramatically affect cellular cholesterol visualisation with implications for assessing cholesterol levels and localisation across the lysosomal disorders. Presented at: 19th Annual WORLDSymposium™ 2023, Orlando, Florida, 21-26 February 2023, Vol. 138. Vol. 2. Elsevier pp. 133., (10.1016/j.ymgme.2022.107352)
- Lloyd-Evans, E., Best, H. L., Alshehri, A. S., Honeybun, L. and Waller-Evans, H. 2023. Glycosphingolipid reduction with miglustat as a therapeutic strategy for CLN3 and other neuronal ceroid lipofuscinoses.. Presented at: 19th Annual WORLDSymposium™ 2023, Orlando, Florida, 21-26 February 2023, Vol. 138. Vol. 2. Elsevier pp. 84., (10.1016/j.ymgme.2022.107212)
- Cook, S. R., Kirkham, E. D., Best, H., Waller-Evans, H., Serpi, M., Pertusati, F. and Lloyd-Evans, E. 2023. Investigating lysosomal and cellular phenotypes of the lysosomal disorder cystinosis. Presented at: 19th Annual WORLDSymposium™ 2023, Orlando, Florida, 21-26 February 2023, Vol. 138. Vol. 2. Elsevier pp. 28-28., (10.1016/j.ymgme.2022.107061)
- Fenn, G. D., Lloyd-Evans, E. and Waller-Evans, H. 2023. Investigation of cannabidiol as a potential therapeutic in Niemann-Pick disease. Presented at: 19th Annual WORLDSymposium™ 2023, Orlando, Florida, 21-26 February 2023, Vol. 138. Vol. 2. Elsevier pp. 42-42., (10.1016/j.ymgme.2022.107098)
- Waller-Evans, H., Zhu, J., Honeybun, L., Gardiner, S., Hanley, M., Best, H. and Lloyd-Evans, E. 2023. Measuring ion flux via lysosomal channels: Primary and secondary assays for drug discovery across the lysosomal disorders. Presented at: 19th Annual WORLDSymposium™ 2023, Orlando, Florida, 21-26 February 2023, Vol. 138. Vol. 2. Elsevier pp. 133-134., (10.1016/j.ymgme.2022.107353)
- Honeybun, L., Waller-Evans, H. and Lloyd-Evans, E. 2023. Unbiased phenotypic drug screen for CLN3 disease. Presented at: 19th Annual WORLDSymposium™ 2023, Orlando, Florida, 21-26 February 2023, Vol. 138. Vol. 2. Elsevier pp. 167-167., (10.1016/j.ymgme.2022.107150)
- Best, H., Honeybun, L., Waller-Evans, H. and Lloyd-Evans, E. 2023. The Batten disease associated protein CLN3 is required for the efflux of lysosomal K. Presented at: 19th Annual WORLDSymposium™ 2023, Orlando, Florida, 21-26 February 2023, Vol. 138. Vol. 2. Elsevier pp. 47-47., (10.1016/j.ymgme.2022.107030)
2022
- Best, H. L., Williamson, L. J., Lipka-Lloyd, M., Waller-Evans, H., Lloyd-Evans, E., Rizkallah, P. J. and Berry, C. 2022. The crystal structure of Bacillus thuringiensis Tpp80Aa1 and its interaction with galactose-containing glycolipids. Toxins 14(12), article number: 863. (10.3390/toxins14120863)
2021
- Maguire, E. et al. 2021. PIP2 depletion and altered endocytosis caused by expression of Alzheimer's disease-protective variant PLCγ2 R522. EMBO Journal 40(17), article number: e105603. (10.15252/embj.2020105603)
2020
- Cook, S. R. et al. 2020. Visualisation of cholesterol and ganglioside GM1 in zebrafish models of Niemann-Pick type C disease and Smith-Lemli-Opitz syndrome using light sheet microscopy. Histochemistry and Cell Biology 154, pp. 565-578. (10.1007/s00418-020-01925-2)
- Badell Grau, R. A., Cuff, J. P., Kelly, B. P., Waller-Evans, H. and Lloyd-Evans, E. 2020. Investigating the prevalence of reactive online searching in the COVID-19 pandemic. Journal of Medical Internet Research 22(10), article number: e19791. (10.2196/19791)
- Lloyd-Evans, E. and Waller-Evans, H. 2020. Biosynthesis and signalling functions of central and peripheral nervous system neurosteroids in health and disease. Essays in Biochemistry 64(3), pp. 591-606. (10.1042/EBC20200043)
- Harwood, J. L. and Lloyd-Evans, E. eds. 2020. Essays in biochemistry volume 64, issue 3: lipid mediators. Portland Press.
- Cook, S. R. et al. 2020. Detrimental effect of zwitterionic buffers on lysosomal homeostasis in cell lines and iPSC-derived neurons. AMRC Open Research 2, article number: 21. (10.12688/amrcopenres.12903.1)
- Day, A. H. et al. 2020. Targeted cell imaging properties of a deep red luminescent iridium(III) complex conjugated with a c-Myc signal peptide. Chemical Science 11(6), pp. 1599-1606. (10.1039/C9SC05568A)
2019
- Carreira, A. et al. 2019. Mammalian sphingoid bases: Biophysical, physiological and pathological properties. Progress in Lipid Research 75, article number: 100988. (10.1016/j.plipres.2019.100988)
- Balogi, Z. et al. 2019. Hsp70 interactions with membrane lipids regulate cellular functions in health and disease. Progress in Lipid Research 74, pp. 18-30. (10.1016/j.plipres.2019.01.004)
2018
- Morimoto, M. et al. 2018. Bi-allelic CCDC47 variants cause a disorder characterized by woolly hair, liver dysfunction, dysmorphic features, and global developmental delay. American Journal of Human Genetics 103(5), pp. 794-807. (10.1016/j.ajhg.2018.09.014)
- Lange, J., Haslett, L. J., Lloyd-Evans, E., Pocock, J. M., Sands, M. S., Williams, B. P. and Cooper, J. D. 2018. Compromised astrocyte function and survival negatively impact neurons in infantile neuronal ceroid lipofuscinosis. Acta Neuropathologica Communications 6, article number: 74. (10.1186/s40478-018-0575-4)
2017
- Fineran, P. et al. 2017. Pathogenic mycobacteria achieve cellular persistence by inhibiting the Niemann-Pick Type C disease cellular pathway. Wellcome Open Research 1, article number: 18. (10.12688/wellcomeopenres.10036.2)
2016
- Lloyd-Evans, E. and Haslett, L. 2016. The lysosomal storage disease continuum with ageing-related neurodegenerative disease. Ageing Research Reviews 32, pp. 104-121. (10.1016/j.arr.2016.07.005)
- Lloyd-Evans, E. 2016. On the move, lysosomal CAX drives Ca2+ transport and motility. Journal of cell biology 212(7), pp. 755-757. (10.1083/jcb.201603037)
2015
- Oswald, M. C. W., West, R. J. H., Lloyd-Evans, E. and Sweeney, S. T. 2015. Identification of dietary alanine toxicity and trafficking dysfunction in a Drosophila model of hereditary sensory and autonomic neuropathy type 1. Human Molecular Genetics 24(24), pp. 6899-6909. (10.1093/hmg/ddv390)
- Lee, J. et al. 2015. Presenilin 1 maintains Lysosomal Ca2+ homeostasis via TRPML1 by regulating vATPase-mediated lysosome acidification. Cell Reports 12(9), pp. 1430-1444. (10.1016/j.celrep.2015.07.050)
- Chandrachud, U. et al. 2015. Unbiased cell-based screening in a neuronal cell model of Batten Disease highlights an interaction between Ca2+Homeostasis, autophagy, and CLN3 protein function. Journal of Biological Chemistry 290(23), pp. 14361-14380. (10.1074/jbc.M114.621706)
- Waller-Evans, H. and Lloyd-Evans, E. 2015. Regulation of TRPML1 function. Biochemical Society Transactions 43(3), pp. 442-446. (10.1042/BST20140311)
- Walker, M. W. and Lloyd-Evans, E. 2015. A rapid method for the preparation of ultrapure, functional lysosomes using functionalized superparamagnetic iron oxide nanoparticles. Methods in Cell Biology 126, pp. 21-43. (10.1016/bs.mcb.2014.10.019)
2014
- Platt, F. M., Wassif, C., Colaco, A., Dardis, A., Lloyd-Evans, E., Bembi, B. and Porter, F. D. 2014. Disorders of cholesterol metabolism and their unanticipated convergent mechanisms of disease. Annual Review of Genomics and Human Genetics 15(1), pp. 173-194. (10.1146/annurev-genom-091212-153412)
2011
- Morgan, A. J., Platt, F. M., Lloyd‑Evans, E. and Galione, A. 2011. Molecular mechanisms of endolysosomal Ca2+ signalling in health and disease. Biochemical Journal 439(3), pp. 349-374. (10.1042/BJ20110949)
- Lloyd-Evans, E. and Platt, F. M. 2011. Lysosomal Ca2+ homeostasis: Role in pathogenesis of lysosomal storage diseases. Cell Calcium 50(2), pp. 200-205. (10.1016/j.ceca.2011.03.010)
2010
- Lloyd-Evans, E., Waller-Evans, H., Peterneva, K. and Platt, F. M. 2010. Endolysosomal calcium regulation and disease.. Biochemical Society Transactions 38(6), pp. 1458-1464. (10.1042/BST0381458)
- Ruas, M. et al. 2010. Purified TPC isoforms form NAADP receptors with distinct roles for Ca2+ signaling and endolysosomal trafficking. Current Biology 20(8), pp. 703-709. (10.1016/j.cub.2010.02.049)
- Lloyd-Evans, E. and Platt, F. M. 2010. Lipids on trial: The search for the offending metabolite in Niemann-Pick type C disease. Traffic 11(4), pp. 419-428. (10.1111/j.1600-0854.2010.01032.x)
2008
- Lloyd-Evans, E. et al. 2008. Niemann-Pick disease type C1 is a sphingosine storage disease that causes deregulation of lysosomal calcium. Nature Medicine 14(11), pp. 1247-1255. (10.1038/nm.1876)
- Elliot-Smith, E. et al. 2008. Beneficial effects of substrate reduction therapy in a mouse model of GM1 gangliosidosis. Molecular Genetics and Metabolism 94(2), pp. 204-211. (10.1016/j.ymgme.2008.02.005)
2005
- Pelled, D., Trajkovic-Bodennec, S., Lloyd-Evans, E., Sidransky, E., Schiffmann, R. and Futerman, A. H. 2005. Enhanced calcium release in the acute neuronopathic form of Gaucher disease. Neurobiology of Disease 18(1), pp. 83-88. (10.1016/j.nbd.2004.09.004)
2004
- Lachmann, R. H. et al. 2004. Treatment with miglustat reverses the lipid-trafficking defect in Niemann–Pick disease type C. Neurobiology of Disease 16(3), pp. 654-658. (10.1016/j.nbd.2004.05.002)
- Vruchte, D. T. et al. 2004. Accumulation of glycosphingolipids in Niemann-Pick C disease disrupts endosomal transport. Journal of Biological Chemistry 279(25), pp. 26167-26175. (10.1074/jbc.M311591200)
2003
- Lloyd-Evans, E., Pelled, D., Riebeling, C. and Futerman, A. H. 2003. Lyso-glycosphingolipids mobilize calcium from brain microsomes via multiple mechanisms. Biochemical Journal 375(3), pp. 561-565. (10.1042/BJ20030613)
- Pelled, D., Lloyd-Evans, E., Riebeling, C., Jeyakumar, M., Platt, F. M. and Futerman, A. H. 2003. Inhibition of calcium uptake via the sarco/endoplasmic reticulum Ca2+-ATPase in a mouse model of Sandhoff disease and prevention by treatment with N-Butyldeoxynojirimycin. Journal of Biological Chemistry 278(32), pp. 29496-29501. (10.1074/jbc.M302964200)
- Lloyd-Evans, E. et al. 2003. Glucosylceramide and glucosylsphingosine modulate calcium mobilization from brain microsomes via different mechanisms. Journal of Biological Chemistry 278(26), pp. 23594-23599. (10.1074/jbc.M300212200)
Articles
- Williamson, L. J. et al. 2023. Structure of the Lysinibacillus sphaericus Tpp49Aa1 pesticidal protein elucidated from natural crystals using MHz-SFX. Proceedings of the National Academy of Sciences 120(49), article number: e2203241120. (10.1073/pnas.2203241120)
- Best, H. L., Williamson, L. J., Heath, E. A., Waller-Evans, H., Lloyd-Evans, E. and Berry, C. 2023. The role of glycoconjugates as receptors for insecticidal proteins. FEMS Microbiology Reviews 47(4) (10.1093/femsre/fuad026)
- Somogyi, A. et al. 2023. The synthetic TRPML1 agonist ML-SA1 rescues Alzheimer-related alterations of the endosomal-autophagic-lysosomal system. Journal of Cell Science 136(6), article number: jcs259875. (10.1242/jcs.259875)
- Fenn, G. D., Alenazy, D. M., Pope, S., Lloyd-Evans, E. and Waller-Evans, H. 2023. Development of a novel anthraquinone-derived fluorescent lysosomal probe. Molecular Genetics and Metabolism 138(2), article number: 107097. (10.1016/j.ymgme.2022.107097)
- Best, H. L., Williamson, L. J., Lipka-Lloyd, M., Waller-Evans, H., Lloyd-Evans, E., Rizkallah, P. J. and Berry, C. 2022. The crystal structure of Bacillus thuringiensis Tpp80Aa1 and its interaction with galactose-containing glycolipids. Toxins 14(12), article number: 863. (10.3390/toxins14120863)
- Maguire, E. et al. 2021. PIP2 depletion and altered endocytosis caused by expression of Alzheimer's disease-protective variant PLCγ2 R522. EMBO Journal 40(17), article number: e105603. (10.15252/embj.2020105603)
- Cook, S. R. et al. 2020. Visualisation of cholesterol and ganglioside GM1 in zebrafish models of Niemann-Pick type C disease and Smith-Lemli-Opitz syndrome using light sheet microscopy. Histochemistry and Cell Biology 154, pp. 565-578. (10.1007/s00418-020-01925-2)
- Badell Grau, R. A., Cuff, J. P., Kelly, B. P., Waller-Evans, H. and Lloyd-Evans, E. 2020. Investigating the prevalence of reactive online searching in the COVID-19 pandemic. Journal of Medical Internet Research 22(10), article number: e19791. (10.2196/19791)
- Lloyd-Evans, E. and Waller-Evans, H. 2020. Biosynthesis and signalling functions of central and peripheral nervous system neurosteroids in health and disease. Essays in Biochemistry 64(3), pp. 591-606. (10.1042/EBC20200043)
- Cook, S. R. et al. 2020. Detrimental effect of zwitterionic buffers on lysosomal homeostasis in cell lines and iPSC-derived neurons. AMRC Open Research 2, article number: 21. (10.12688/amrcopenres.12903.1)
- Day, A. H. et al. 2020. Targeted cell imaging properties of a deep red luminescent iridium(III) complex conjugated with a c-Myc signal peptide. Chemical Science 11(6), pp. 1599-1606. (10.1039/C9SC05568A)
- Carreira, A. et al. 2019. Mammalian sphingoid bases: Biophysical, physiological and pathological properties. Progress in Lipid Research 75, article number: 100988. (10.1016/j.plipres.2019.100988)
- Balogi, Z. et al. 2019. Hsp70 interactions with membrane lipids regulate cellular functions in health and disease. Progress in Lipid Research 74, pp. 18-30. (10.1016/j.plipres.2019.01.004)
- Morimoto, M. et al. 2018. Bi-allelic CCDC47 variants cause a disorder characterized by woolly hair, liver dysfunction, dysmorphic features, and global developmental delay. American Journal of Human Genetics 103(5), pp. 794-807. (10.1016/j.ajhg.2018.09.014)
- Lange, J., Haslett, L. J., Lloyd-Evans, E., Pocock, J. M., Sands, M. S., Williams, B. P. and Cooper, J. D. 2018. Compromised astrocyte function and survival negatively impact neurons in infantile neuronal ceroid lipofuscinosis. Acta Neuropathologica Communications 6, article number: 74. (10.1186/s40478-018-0575-4)
- Fineran, P. et al. 2017. Pathogenic mycobacteria achieve cellular persistence by inhibiting the Niemann-Pick Type C disease cellular pathway. Wellcome Open Research 1, article number: 18. (10.12688/wellcomeopenres.10036.2)
- Lloyd-Evans, E. and Haslett, L. 2016. The lysosomal storage disease continuum with ageing-related neurodegenerative disease. Ageing Research Reviews 32, pp. 104-121. (10.1016/j.arr.2016.07.005)
- Lloyd-Evans, E. 2016. On the move, lysosomal CAX drives Ca2+ transport and motility. Journal of cell biology 212(7), pp. 755-757. (10.1083/jcb.201603037)
- Oswald, M. C. W., West, R. J. H., Lloyd-Evans, E. and Sweeney, S. T. 2015. Identification of dietary alanine toxicity and trafficking dysfunction in a Drosophila model of hereditary sensory and autonomic neuropathy type 1. Human Molecular Genetics 24(24), pp. 6899-6909. (10.1093/hmg/ddv390)
- Lee, J. et al. 2015. Presenilin 1 maintains Lysosomal Ca2+ homeostasis via TRPML1 by regulating vATPase-mediated lysosome acidification. Cell Reports 12(9), pp. 1430-1444. (10.1016/j.celrep.2015.07.050)
- Chandrachud, U. et al. 2015. Unbiased cell-based screening in a neuronal cell model of Batten Disease highlights an interaction between Ca2+Homeostasis, autophagy, and CLN3 protein function. Journal of Biological Chemistry 290(23), pp. 14361-14380. (10.1074/jbc.M114.621706)
- Waller-Evans, H. and Lloyd-Evans, E. 2015. Regulation of TRPML1 function. Biochemical Society Transactions 43(3), pp. 442-446. (10.1042/BST20140311)
- Walker, M. W. and Lloyd-Evans, E. 2015. A rapid method for the preparation of ultrapure, functional lysosomes using functionalized superparamagnetic iron oxide nanoparticles. Methods in Cell Biology 126, pp. 21-43. (10.1016/bs.mcb.2014.10.019)
- Platt, F. M., Wassif, C., Colaco, A., Dardis, A., Lloyd-Evans, E., Bembi, B. and Porter, F. D. 2014. Disorders of cholesterol metabolism and their unanticipated convergent mechanisms of disease. Annual Review of Genomics and Human Genetics 15(1), pp. 173-194. (10.1146/annurev-genom-091212-153412)
- Morgan, A. J., Platt, F. M., Lloyd‑Evans, E. and Galione, A. 2011. Molecular mechanisms of endolysosomal Ca2+ signalling in health and disease. Biochemical Journal 439(3), pp. 349-374. (10.1042/BJ20110949)
- Lloyd-Evans, E. and Platt, F. M. 2011. Lysosomal Ca2+ homeostasis: Role in pathogenesis of lysosomal storage diseases. Cell Calcium 50(2), pp. 200-205. (10.1016/j.ceca.2011.03.010)
- Lloyd-Evans, E., Waller-Evans, H., Peterneva, K. and Platt, F. M. 2010. Endolysosomal calcium regulation and disease.. Biochemical Society Transactions 38(6), pp. 1458-1464. (10.1042/BST0381458)
- Ruas, M. et al. 2010. Purified TPC isoforms form NAADP receptors with distinct roles for Ca2+ signaling and endolysosomal trafficking. Current Biology 20(8), pp. 703-709. (10.1016/j.cub.2010.02.049)
- Lloyd-Evans, E. and Platt, F. M. 2010. Lipids on trial: The search for the offending metabolite in Niemann-Pick type C disease. Traffic 11(4), pp. 419-428. (10.1111/j.1600-0854.2010.01032.x)
- Lloyd-Evans, E. et al. 2008. Niemann-Pick disease type C1 is a sphingosine storage disease that causes deregulation of lysosomal calcium. Nature Medicine 14(11), pp. 1247-1255. (10.1038/nm.1876)
- Elliot-Smith, E. et al. 2008. Beneficial effects of substrate reduction therapy in a mouse model of GM1 gangliosidosis. Molecular Genetics and Metabolism 94(2), pp. 204-211. (10.1016/j.ymgme.2008.02.005)
- Pelled, D., Trajkovic-Bodennec, S., Lloyd-Evans, E., Sidransky, E., Schiffmann, R. and Futerman, A. H. 2005. Enhanced calcium release in the acute neuronopathic form of Gaucher disease. Neurobiology of Disease 18(1), pp. 83-88. (10.1016/j.nbd.2004.09.004)
- Lachmann, R. H. et al. 2004. Treatment with miglustat reverses the lipid-trafficking defect in Niemann–Pick disease type C. Neurobiology of Disease 16(3), pp. 654-658. (10.1016/j.nbd.2004.05.002)
- Vruchte, D. T. et al. 2004. Accumulation of glycosphingolipids in Niemann-Pick C disease disrupts endosomal transport. Journal of Biological Chemistry 279(25), pp. 26167-26175. (10.1074/jbc.M311591200)
- Lloyd-Evans, E., Pelled, D., Riebeling, C. and Futerman, A. H. 2003. Lyso-glycosphingolipids mobilize calcium from brain microsomes via multiple mechanisms. Biochemical Journal 375(3), pp. 561-565. (10.1042/BJ20030613)
- Pelled, D., Lloyd-Evans, E., Riebeling, C., Jeyakumar, M., Platt, F. M. and Futerman, A. H. 2003. Inhibition of calcium uptake via the sarco/endoplasmic reticulum Ca2+-ATPase in a mouse model of Sandhoff disease and prevention by treatment with N-Butyldeoxynojirimycin. Journal of Biological Chemistry 278(32), pp. 29496-29501. (10.1074/jbc.M302964200)
- Lloyd-Evans, E. et al. 2003. Glucosylceramide and glucosylsphingosine modulate calcium mobilization from brain microsomes via different mechanisms. Journal of Biological Chemistry 278(26), pp. 23594-23599. (10.1074/jbc.M300212200)
Books
- Harwood, J. L. and Lloyd-Evans, E. eds. 2020. Essays in biochemistry volume 64, issue 3: lipid mediators. Portland Press.
Conferences
- Cook, S. R. and Lloyd-Evans, E. 2023. Comparing the lysosomal biotoxicity of iron oxide nanoparticles for improved lysosomal purification. Presented at: 19th Annual WORLDSymposiumTM, Orlando, Florida, 21-26 February 2023, Vol. 138. Vol. 2. Elsevier pp. 28-29., (10.1016/j.ymgme.2022.107062)
- Lloyd-Evans, E. and Evans, C. 2023. Deciphering the various mechanisms of action of miglustat in the lysosomal disorders.. Presented at: 19th Annual WORLDSymposiumTM, Orlando, Florida, 21-26 February 2023, Vol. 138. Vol. 2. Elsevier pp. 84., (10.1016/j.ymgme.2022.107213)
- Best, H. L., Badell-Grau, R. A., Shipley, K., Alshehri, A. S., Waller-Evans, H. and Lloyd-Evans, E. 2023. Disease phenotypes present in CLN7 patient fibroblasts are improved following treatment to reduce glycosphingolipid synthesis.. Presented at: 19th Annual WORLDSymposium™ 2023, Orlando, Florida, 21-26 February 2023, Vol. 138. Vol. 2. Elsevier pp. 17., (10.1016/j.ymgme.2022.107029)
- Waller-Evans, H., Francis, L., Fenn, G., Best, H. and Lloyd-Evans, E. 2023. Filipin composition and imaging modality dramatically affect cellular cholesterol visualisation with implications for assessing cholesterol levels and localisation across the lysosomal disorders. Presented at: 19th Annual WORLDSymposium™ 2023, Orlando, Florida, 21-26 February 2023, Vol. 138. Vol. 2. Elsevier pp. 133., (10.1016/j.ymgme.2022.107352)
- Lloyd-Evans, E., Best, H. L., Alshehri, A. S., Honeybun, L. and Waller-Evans, H. 2023. Glycosphingolipid reduction with miglustat as a therapeutic strategy for CLN3 and other neuronal ceroid lipofuscinoses.. Presented at: 19th Annual WORLDSymposium™ 2023, Orlando, Florida, 21-26 February 2023, Vol. 138. Vol. 2. Elsevier pp. 84., (10.1016/j.ymgme.2022.107212)
- Cook, S. R., Kirkham, E. D., Best, H., Waller-Evans, H., Serpi, M., Pertusati, F. and Lloyd-Evans, E. 2023. Investigating lysosomal and cellular phenotypes of the lysosomal disorder cystinosis. Presented at: 19th Annual WORLDSymposium™ 2023, Orlando, Florida, 21-26 February 2023, Vol. 138. Vol. 2. Elsevier pp. 28-28., (10.1016/j.ymgme.2022.107061)
- Fenn, G. D., Lloyd-Evans, E. and Waller-Evans, H. 2023. Investigation of cannabidiol as a potential therapeutic in Niemann-Pick disease. Presented at: 19th Annual WORLDSymposium™ 2023, Orlando, Florida, 21-26 February 2023, Vol. 138. Vol. 2. Elsevier pp. 42-42., (10.1016/j.ymgme.2022.107098)
- Waller-Evans, H., Zhu, J., Honeybun, L., Gardiner, S., Hanley, M., Best, H. and Lloyd-Evans, E. 2023. Measuring ion flux via lysosomal channels: Primary and secondary assays for drug discovery across the lysosomal disorders. Presented at: 19th Annual WORLDSymposium™ 2023, Orlando, Florida, 21-26 February 2023, Vol. 138. Vol. 2. Elsevier pp. 133-134., (10.1016/j.ymgme.2022.107353)
- Honeybun, L., Waller-Evans, H. and Lloyd-Evans, E. 2023. Unbiased phenotypic drug screen for CLN3 disease. Presented at: 19th Annual WORLDSymposium™ 2023, Orlando, Florida, 21-26 February 2023, Vol. 138. Vol. 2. Elsevier pp. 167-167., (10.1016/j.ymgme.2022.107150)
- Best, H., Honeybun, L., Waller-Evans, H. and Lloyd-Evans, E. 2023. The Batten disease associated protein CLN3 is required for the efflux of lysosomal K. Presented at: 19th Annual WORLDSymposium™ 2023, Orlando, Florida, 21-26 February 2023, Vol. 138. Vol. 2. Elsevier pp. 47-47., (10.1016/j.ymgme.2022.107030)
Ymchwil
Our current interests
- Mechanisms regulating lysosomal calcium ion (Ca2+) and zinc ion (Zn2+) homeostasis in health and disease
- Use of superparamagnetic ferrofluid to purify lysosomes
- Function of the NPC1 protein as a lysosomal RND multi-substrate permease and it's role in tuberculosis
- Sterol precursor inhibition of the NPC1 protein as a therapeutic target for the sterol biosynthetic disease Smith-Lemli-Opitz Syndrome
- Role of lysosomal dysfunction in the pathogenesis of Parkinson's disease
- Mechanisms of pathogenesis and therapy development for soluble lysosomal protein diseases (MPS type II, NPC2, Tay-Sachs)
Group members
- Mr Luke Haslett (Research Associate)
- Charlie De Haviland (PTY student)
- Miss Emily Kirkham (Research Assistant & PhD student)
- Louisa Smither (PTY student)
- Dr Helen Waller-Evans (Research Associate)
- Mr Abdullah Alshehri (PhD student)
- Mr Rafael Andres Badell Grau (PhD student)
- Miss Ana Carreira (joint PhD student with Dr. Liana Silva, Faculda Farmacia, University of Lisbon)
- Miss Emily Clark (PhD student)
- Miss Emily Maguire (PhD student)
- Miss Katie Shipley (PhD student)
- Mr Jordan Copner (undergraduate placement student)
Bywgraffiad
Fy ngradd gyntaf oedd gradd israddedig mewn Biocemeg (M.Biochem) ym Mhrifysgol Caerfaddon. Yn ystod y radd hon, treuliais 11 mis (2 leoliad yn olynol) yn labordy yr Athro Tony Futerman yn Sefydliad Gwyddoniaeth Weizmann, Rehovot, Israel. Yma y cefais ddiddordeb mewn clefydau storio lysosomal, gan ymchwilio i rôl homeostasis endoplasmig (homeostasis) Ca2+ endoplasmig wedi'i newid mewn clefyd Gaucher. Yn dilyn fy ngradd yn 2002 symudais i Rydychen i wneud fy DPhil gyda'r Athro Fran Platt yn y Sefydliad Glycobiology. Yma, ymchwiliais i rôl y sffingosîn sffingolipid syml ym bathogenesis Niemann-Pick math C1. Ar ôl cwblhau fy DPhil Yn 2005, symudais gyda Fran i'r Adran Ffarmacoleg (Rhydychen) lle, mewn cydweithrediad â'r Athro Antony Galione, datblygom dechnegau i astudio homeostasis lysosomal Ca2+ yn y clefydau lysosomal. Yn 2010 cefais fy mhenodi'n Gymrawd RCUK yn Ysgol y Biowyddorau, Prifysgol Caerdydd, i barhau â'm hymchwil i swyddogaeth lysosomaidd.
