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Lipids on Trial: The Search for the Offending Metabolite in Niemann-Pick type C Disease

Traffic (Copenhagen, Denmark), 2010-04, Vol.11 (4), p.419-428

2010

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PIP2 depletion and altered endocytosis caused by expression of Alzheimer's disease‐protective variant PLCγ2 R522

The EMBO journal, 2021-09, Vol.40 (17), p.e105603-n/a

2021

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Endolysosomal calcium regulation and disease

Biochemical Society transactions, 2010-12, Vol.38 (6), p.1458-1464

2010

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Presenilin 1 Maintains Lysosomal Ca2+ Homeostasis via TRPML1 by Regulating vATPase-Mediated Lysosome Acidification

Cell reports (Cambridge), 2015-09, Vol.12 (9), p.1430-1444

2015

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Regulation of TRPML1 function

Biochemical Society transactions, 2015-06, Vol.43 (3), p.442-446

2015

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Unbiased Cell-based Screening in a Neuronal Cell Model of Batten Disease Highlights an Interaction between Ca2+ Homeostasis, Autophagy, and CLN3 Protein Function

The Journal of biological chemistry, 2015-06, Vol.290 (23), p.14361-14380

2015

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A rapid method for the preparation of ultrapure, functional lysosomes using functionalized superparamagnetic iron oxide nanoparticles

Methods in cell biology, 2015, Vol.126, p.21-43

2015

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The Crystal Structure of Bacillus thuringiensis Tpp80Aa1 and Its Interaction with Galactose-Containing Glycolipids

Toxins, 2022-12, Vol.14 (12), p.863

2022

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Compromised astrocyte function and survival negatively impact neurons in infantile neuronal ceroid lipofuscinosis

Acta neuropathologica communications, 2018-08, Vol.6 (1), p.74-74, Article 74

2018

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Enhanced calcium release in the acute neuronopathic form of Gaucher disease

Neurobiology of disease, 2005-02, Vol.18 (1), p.83-88

2005

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Glucosylceramide and Glucosylsphingosine Modulate Calcium Mobilization from Brain Microsomes via Different Mechanisms

The Journal of biological chemistry, 2003-06, Vol.278 (26), p.23594-23599

2003

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Accumulation of Glycosphingolipids in Niemann-Pick C Disease Disrupts Endosomal Transport

The Journal of biological chemistry, 2004-06, Vol.279 (25), p.26167-26175

2004

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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

The Journal of biological chemistry, 2003-08, Vol.278 (32), p.29496-29501

2003

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Lysosomal Ca2+ Homeostasis and Signaling in Health and Disease

Cold Spring Harbor perspectives in biology, 2020-06, Vol.12 (6), p.1

2020

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Treatment with miglustat reverses the lipid-trafficking defect in Niemann–Pick disease type C

Neurobiology of disease, 2004-08, Vol.16 (3), p.654-658

2004

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Pathogenic mycobacteria achieve cellular persistence by inhibiting the Niemann-Pick Type C disease cellular pathway

Wellcome open research, 2017-06, Vol.1, p.18-18

2017

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Deciphering the various mechanisms of action of miglustat in the lysosomal disorders

Molecular genetics and metabolism, 2023-02, Vol.138 (2), p.107213, Article 107213

2023

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Detrimental effect of zwitterionic buffers on lysosomal homeostasis in cell lines and iPSC-derived neurons

AMRC open research, 2020-05, Vol.2, p.21

2020

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Beneficial effects of substrate reduction therapy in a mouse model of GM1 gangliosidosis

Molecular genetics and metabolism, 2008-06, Vol.94 (2), p.204-211

2008

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Biosynthesis and signalling functions of central and peripheral nervous system neurosteroids in health and disease

Essays in biochemistry, 2020-09, Vol.64 (3), p.591-606

2020

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Pathogenic mycobacteria achieve cellular persistence by inhibiting the Niemann-Pick Type C disease cellular pathway

Wellcome open research, 2017, Vol.1, p.18

2017

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On the move, lysosomal CAX drives Ca2+ transport and motility

The Journal of cell biology, 2016-03, Vol.212 (7), p.755-757

Lloyd-Evans, Emyr

2016

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Comparing the lysosomal biotoxicity of iron oxide nanoparticles for improved lysosomal purification

Molecular genetics and metabolism, 2023-02, Vol.138 (2), p.107062, Article 107062

2023

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On the move, lysosomal CAX drives Ca^sup 2+^ transport and motility

The Journal of cell biology, 2016-03, Vol.212 (7), p.755

Lloyd-Evans, Emyr

2016

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Molecular mechanisms of endolysosomal Ca2+ signalling in health and disease

Biochemical journal, 2011-11, Vol.439 (3), p.349-378

2011

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Lipids on Trial: The Search for the Offending Metabolite in Niemann-Pick type C Disease

PIP2 depletion and altered endocytosis caused by expression of Alzheimer's disease‐protective variant PLCγ2 R522

Endolysosomal calcium regulation and disease

Presenilin 1 Maintains Lysosomal Ca2+ Homeostasis via TRPML1 by Regulating vATPase-Mediated Lysosome Acidification

Regulation of TRPML1 function

Unbiased Cell-based Screening in a Neuronal Cell Model of Batten Disease Highlights an Interaction between Ca2+ Homeostasis, Autophagy, and CLN3 Protein Function

A rapid method for the preparation of ultrapure, functional lysosomes using functionalized superparamagnetic iron oxide nanoparticles

The Crystal Structure of Bacillus thuringiensis Tpp80Aa1 and Its Interaction with Galactose-Containing Glycolipids

Compromised astrocyte function and survival negatively impact neurons in infantile neuronal ceroid lipofuscinosis

Enhanced calcium release in the acute neuronopathic form of Gaucher disease

Glucosylceramide and Glucosylsphingosine Modulate Calcium Mobilization from Brain Microsomes via Different Mechanisms

Accumulation of Glycosphingolipids in Niemann-Pick C Disease Disrupts Endosomal Transport

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

Lysosomal Ca2+ Homeostasis and Signaling in Health and Disease

Treatment with miglustat reverses the lipid-trafficking defect in Niemann–Pick disease type C

Pathogenic mycobacteria achieve cellular persistence by inhibiting the Niemann-Pick Type C disease cellular pathway

Deciphering the various mechanisms of action of miglustat in the lysosomal disorders

Detrimental effect of zwitterionic buffers on lysosomal homeostasis in cell lines and iPSC-derived neurons

Beneficial effects of substrate reduction therapy in a mouse model of GM1 gangliosidosis

Biosynthesis and signalling functions of central and peripheral nervous system neurosteroids in health and disease

Pathogenic mycobacteria achieve cellular persistence by inhibiting the Niemann-Pick Type C disease cellular pathway

On the move, lysosomal CAX drives Ca2+ transport and motility

Comparing the lysosomal biotoxicity of iron oxide nanoparticles for improved lysosomal purification

On the move, lysosomal CAX drives Ca^sup 2+^ transport and motility

Molecular mechanisms of endolysosomal Ca2+ signalling in health and disease

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