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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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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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Investigation of cannabidiol as a potential therapeutic in Niemann-Pick disease

Molecular genetics and metabolism, 2023-02, Vol.138 (2), p.107098, Article 107098

2023

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The Batten disease associated protein CLN3 is required for the efflux of lysosomal K

Molecular genetics and metabolism, 2023-02, Vol.138 (2), p.107030, Article 107030

2023

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Unbiased phenotypic drug screen for CLN3 disease

Molecular genetics and metabolism, 2023-02, Vol.138 (2), p.107150, Article 107150

2023

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Hsp70 interactions with membrane lipids regulate cellular functions in health and disease

Progress in lipid research, 2019-04, Vol.74, p.18-30

2019

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Lysosomal Ca2+ homeostasis: Role in pathogenesis of lysosomal storage diseases

Cell calcium (Edinburgh), 2011-08, Vol.50 (2), p.200-205

2011

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The lysosomal storage disease continuum with ageing-related neurodegenerative disease

Ageing research reviews, 2016-12, Vol.32, p.104-121

2016

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Lysosomal Ca(2+) homeostasis: role in pathogenesis of lysosomal storage diseases

Cell calcium (Edinburgh), 2011-08, Vol.50 (2), p.200-205

2011

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Filipin composition and imaging modality dramatically affect cellular cholesterol visualisation with implications for assessing cholesterol levels and localisation across the lysosomal disorders

Molecular genetics and metabolism, 2023-02, Vol.138 (2), p.107352, Article 107352

2023

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Glycosphingolipid reduction with miglustat as a therapeutic strategy for CLN3 and other neuronal ceroid lipofuscinoses

Molecular genetics and metabolism, 2023-02, Vol.138 (2), p.107212, Article 107212

2023

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Measuring ion flux via lysosomal channels: Primary and secondary assays for drug discovery across the lysosomal disorders

Molecular genetics and metabolism, 2023-02, Vol.138 (2), p.107353, Article 107353

2023

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Development of a novel anthraquinone-derived fluorescent lysosomal probe

Molecular genetics and metabolism, 2023-02, Vol.138 (2), p.107097, Article 107097

2023

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Investigating lysosomal and cellular phenotypes of the lysosomal disorder cystinosis

Molecular genetics and metabolism, 2023-02, Vol.138 (2), p.107061, Article 107061

2023

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Disease phenotypes present in CLN7 patient fibroblasts are improved following treatment to reduce glycosphingolipid synthesis

Molecular genetics and metabolism, 2023-02, Vol.138 (2), p.107029, Article 107029

2023

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Characterisation of the oldest inbred mouse strain, DBA/2j, as a bona fide adult onset model of GM1 gangliosidosis caused by mis-localisation of β-galactosidase

Molecular genetics and metabolism, 2014-02, Vol.111 (2), p.S69-S69

2014

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A new method utilising superparamagnetic magnetite for simple and rapid purification of ultra pure, high yield, functional lysosomes from lysosomal disease cells

Molecular genetics and metabolism, 2014-02, Vol.111 (2), p.S68-S69

2014

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Purified TPC isoforms form NAADP receptors with distinct roles for Ca(2+) signaling and endolysosomal trafficking

Current biology, 2010-04, Vol.20 (8), p.703-709

2010

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Purified TPC Isoforms Form NAADP Receptors with Distinct Roles for Ca2+ Signaling and Endolysosomal Trafficking

Current biology, 2010-04, Vol.20 (8), p.703-709

2010

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Simple fluorimetric test for lysosomal swelling as a means to identify, monitor and develop therapies for all lysosomal diseases

Molecular genetics and metabolism, 2018-02, Vol.123 (2), p.S19-S19

2018

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Biophysical impact of sphingosine and other abnormal lipid accumulation in Niemann-Pick disease type C cell models

Biochimica et biophysica acta. Molecular and cell biology of lipids, 2021-08, Vol.1866 (8), p.158944, Article 158944

2021

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Uncovering the mechanism by which miglustat mediates benefit in NPC disease; role of acid sphingomyelinase in NPC disease pathogenesis

Molecular genetics and metabolism, 2014-02, Vol.111 (2), p.S69-S70

2014

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Lysosomal Ca 2+ homeostasis: Role in pathogenesis of lysosomal storage diseases

Cell calcium (Edinburgh), 2011, Vol.50 (2), p.200-205

2011

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Uncovering the role of lysosomal Ca2 + signalling in two neurodegenerative lysosomal disorders, Niemann-Pick disease type C and mucolipidosis type IV

Molecular genetics and metabolism, 2014-02, Vol.111 (2), p.S70-S70

2014

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Niemann–Pick type C1—A primary sphingosine storage disease?

Chemistry and physics of lipids, 2007-09, Vol.149, p.S18-S19

2007

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

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

Investigation of cannabidiol as a potential therapeutic in Niemann-Pick disease

The Batten disease associated protein CLN3 is required for the efflux of lysosomal K

Unbiased phenotypic drug screen for CLN3 disease

Hsp70 interactions with membrane lipids regulate cellular functions in health and disease

Lysosomal Ca2+ homeostasis: Role in pathogenesis of lysosomal storage diseases

The lysosomal storage disease continuum with ageing-related neurodegenerative disease

Lysosomal Ca(2+) homeostasis: role in pathogenesis of lysosomal storage diseases

Filipin composition and imaging modality dramatically affect cellular cholesterol visualisation with implications for assessing cholesterol levels and localisation across the lysosomal disorders

Glycosphingolipid reduction with miglustat as a therapeutic strategy for CLN3 and other neuronal ceroid lipofuscinoses

Measuring ion flux via lysosomal channels: Primary and secondary assays for drug discovery across the lysosomal disorders

Development of a novel anthraquinone-derived fluorescent lysosomal probe

Investigating lysosomal and cellular phenotypes of the lysosomal disorder cystinosis

Disease phenotypes present in CLN7 patient fibroblasts are improved following treatment to reduce glycosphingolipid synthesis

Characterisation of the oldest inbred mouse strain, DBA/2j, as a bona fide adult onset model of GM1 gangliosidosis caused by mis-localisation of β-galactosidase

A new method utilising superparamagnetic magnetite for simple and rapid purification of ultra pure, high yield, functional lysosomes from lysosomal disease cells

Purified TPC isoforms form NAADP receptors with distinct roles for Ca(2+) signaling and endolysosomal trafficking

Purified TPC Isoforms Form NAADP Receptors with Distinct Roles for Ca2+ Signaling and Endolysosomal Trafficking

Simple fluorimetric test for lysosomal swelling as a means to identify, monitor and develop therapies for all lysosomal diseases

Biophysical impact of sphingosine and other abnormal lipid accumulation in Niemann-Pick disease type C cell models

Uncovering the mechanism by which miglustat mediates benefit in NPC disease; role of acid sphingomyelinase in NPC disease pathogenesis

Lysosomal Ca 2+ homeostasis: Role in pathogenesis of lysosomal storage diseases

Uncovering the role of lysosomal Ca2 + signalling in two neurodegenerative lysosomal disorders, Niemann-Pick disease type C and mucolipidosis type IV

Niemann–Pick type C1—A primary sphingosine storage disease?

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