ArticleRedox biology2017
Mitochondrial GSH replenishment as a potential therapeutic approach for Niemann Pick type C disease.
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Article in Redox biology, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 40 papers.
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40 citing papers in PubMed, 70 citations in OpenAlex.
- Mito-TEMPO improves survival rates inScience advances · 2026Article
- Understanding Liver and Digestive Diseases: A Paved Road to Improve Diagnosis, Management, and Treatment.Exploration of digestive diseases · 2026Article
- l-Cysteine-Glutathione Mixed Disulfide, a Novel Bioavailable Sulfhydryl-Modified Glutathione Precursor, Protects against Early Liver Injury Induced by Short-Term Hypercholesterolemia.Chemical research in toxicology · 2025Article
- Lysosome-Mitochondrial Crosstalk in Cellular Stress and Disease.Antioxidants (Basel, Switzerland) · 2025Review
- Role of lipids in interorganelle communication.Trends in cell biology · 2025Review
- Review
- Global and Targeted Metabolomics for Revealing Metabolomic Alteration in Niemann-Pick Disease Type C Model Cells.Metabolites · 2024Article
- Potential Use of the Cholesterol Transfer Inhibitor U18666A as a Potent Research Tool for the Study of Cholesterol Mechanisms in Neurodegenerative Disorders.Molecular neurobiology · 2024Review
- Advances in research on potential therapeutic approaches for Niemann-Pick C1 disease.Frontiers in pharmacology · 2024Review
- Statins suppress cell-to-cell propagation of α-synuclein by lowering cholesterol.Cell death & disease · 2023Article
- Glutathione Fluorescence Sensing Based on a Co-Doped Carbon Dot/Manganese Dioxide Nanocoral Composite.Materials (Basel, Switzerland) · 2022Article
- Secondary Mitochondrial Dysfunction as a Cause of Neurodegenerative Dysfunction in Lysosomal Storage Diseases and an Overview of Potential Therapies.International journal of molecular sciences · 2022Review
- Metabolic Alteration Analysis of Steroid Hormones in Niemann-Pick Disease Type C Model Cell Using Liquid Chromatography/Tandem Mass Spectrometry.International journal of molecular sciences · 2022Article
- GST-Perfringolysin O production for the localization and quantification of membrane cholesterol in human and mouse brain and liver.STAR protocols · 2022Article
- "Turn on" Fluorescence Sensor of Glutathione Based on Inner Filter Effect of Co-Doped Carbon Dot/Gold Nanoparticle Composites.International journal of molecular sciences · 2021Article
- Cerebellar Long Noncoding RNA Expression Profile in a Niemann-Pick C Disease Mouse Model.Molecular neurobiology · 2021Article
- Acid ceramidase improves mitochondrial function and oxidative stress in Niemann-Pick type C disease by repressing STARD1 expression and mitochondrial cholesterol accumulation.Redox biology · 2021Article
- Article
- Myelin Defects in Niemann-Pick Type C Disease: Mechanisms and Possible Therapeutic Perspectives.International journal of molecular sciences · 2021Review
- Glutathione Metabolism and the Novel Role of Mitochondrial GSH in Retinal Degeneration.Antioxidants (Basel, Switzerland) · 2021Review
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Authors and funding
19 authors at 10 institutions in 4 countries.
Sandra TorresCell Death and Proliferation, Institute of Biomedical Research of Barcelona (IIBB), CSIC, Barcelona, Spain; Liver Unit, Hospital Clinic I Provincial de Barcelona, IDIBAPS and CIBERehd, Barcelona, Spain.
Nuria MatíasCell Death and Proliferation, Institute of Biomedical Research of Barcelona (IIBB), CSIC, Barcelona, Spain; Liver Unit, Hospital Clinic I Provincial de Barcelona, IDIBAPS and CIBERehd, Barcelona, Spain.
Anna BauliesCell Death and Proliferation, Institute of Biomedical Research of Barcelona (IIBB), CSIC, Barcelona, Spain; Liver Unit, Hospital Clinic I Provincial de Barcelona, IDIBAPS and CIBERehd, Barcelona, Spain.
Susana NuñezCell Death and Proliferation, Institute of Biomedical Research of Barcelona (IIBB), CSIC, Barcelona, Spain; Liver Unit, Hospital Clinic I Provincial de Barcelona, IDIBAPS and CIBERehd, Barcelona, Spain.
Cristina Alarcon-VilaCell Death and Proliferation, Institute of Biomedical Research of Barcelona (IIBB), CSIC, Barcelona, Spain; Liver Unit, Hospital Clinic I Provincial de Barcelona, IDIBAPS and CIBERehd, Barcelona, Spain.
Laura MartinezCell Death and Proliferation, Institute of Biomedical Research of Barcelona (IIBB), CSIC, Barcelona, Spain; Liver Unit, Hospital Clinic I Provincial de Barcelona, IDIBAPS and CIBERehd, Barcelona, Spain.
Natalia NuñoCell Death and Proliferation, Institute of Biomedical Research of Barcelona (IIBB), CSIC, Barcelona, Spain; Liver Unit, Hospital Clinic I Provincial de Barcelona, IDIBAPS and CIBERehd, Barcelona, Spain.
Anna FernandezCell Death and Proliferation, Institute of Biomedical Research of Barcelona (IIBB), CSIC, Barcelona, Spain; Liver Unit, Hospital Clinic I Provincial de Barcelona, IDIBAPS and CIBERehd, Barcelona, Spain.
Joan CaballeriaLiver Unit, Hospital Clinic I Provincial de Barcelona, IDIBAPS and CIBERehd, Barcelona, Spain.
Thierry LevadeInstitut National de la Santé et de la Recherche Médicale (INSERM) UMR1037, Centre de Recherches en Cancerologie de Toulouse, Toulouse, France.
Alba Gonzalez-FranquesaDiabetes and Obesity Research Laboratory, Institut d'Investigacions Biomediques August Pi i Sunyer (IDIBAPS) and Spanish Biomedical Research Centre in Diabetes and Associated Metabolic Disorders (CIBERDEM), Barcelona, Spain.
Pablo Garcia-RovésDiabetes and Obesity Research Laboratory, Institut d'Investigacions Biomediques August Pi i Sunyer (IDIBAPS) and Spanish Biomedical Research Centre in Diabetes and Associated Metabolic Disorders (CIBERDEM), Barcelona, Spain.
Elisa BalboaDepartamento de Gastroenterología, Facultad de Medicina, Pontificia Universidad Católica de Chile, Santiago, Chile.
Silvana ZanlungoDepartamento de Gastroenterología, Facultad de Medicina, Pontificia Universidad Católica de Chile, Santiago, Chile.
Gemma FabríasResearch Unit on BioActive Molecules (RUBAM), Departament de Química Orgànica Biològica, Institut d'Investigacions Químiques i Ambientals de Barcelona, Consejo Superior de Investigaciones Científicas (CSIC), Barcelona, Spain.
Josefina CasasResearch Unit on BioActive Molecules (RUBAM), Departament de Química Orgànica Biològica, Institut d'Investigacions Químiques i Ambientals de Barcelona, Consejo Superior de Investigaciones Científicas (CSIC), Barcelona, Spain.
Carlos EnrichCentre de Recerca Biomèdica CELLEX, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), 08036 Barcelona, Spain; Departament de Biologia Cel·lular, Immunologia i Neurociències, Facultat de Medicina, Universitat de Barcelona, 08036 Barcelona, Spain.
Carmen Garcia-RuizCell Death and Proliferation, Institute of Biomedical Research of Barcelona (IIBB), CSIC, Barcelona, Spain; Liver Unit, Hospital Clinic I Provincial de Barcelona, IDIBAPS and CIBERehd, Barcelona, Spain; Research Center for ALPD, Keck School of Medicine, University of Southern California, Los Angeles, CA, United States. Electronic address: cgrbam@iibb.csic.es.
José C Fernández-ChecaCell Death and Proliferation, Institute of Biomedical Research of Barcelona (IIBB), CSIC, Barcelona, Spain; Liver Unit, Hospital Clinic I Provincial de Barcelona, IDIBAPS and CIBERehd, Barcelona, Spain; Research Center for ALPD, Keck School of Medicine, University of Southern California, Los Angeles, CA, United States. Electronic address: checa229@yahoo.com.
Consorci Institut D'Investigacions Biomediques August Pi I Sunyer · ESInstitut d'Investigacions Biomèdiques de Barcelona · ESHospital Clínic de Barcelona · ESPontificia Universidad Católica de Chile · CLCentre de Recherche en Cancérologie de Toulouse · FRCentro de Investigación Biomédica en Red de Enfermedades Hepáticas y Digestivas · ESConsejo Superior de Investigaciones Científicas · ESInstituto de Investigaciones Químicas y Ambientales de Barcelona · ESSpanish Biomedical Research Centre in Physiopathology of Obesity and Nutrition · ESUniversitat de Barcelona · ES
Funding
No grant is acknowledged in the PubMed record.
Abstract
Niemann Pick type C (NPC) disease is a progressive lysosomal storage disorder caused by mutations in genes encoding NPC1/NPC2 proteins, characterized by neurological defects, hepatosplenomegaly and premature death. While the primary biochemical feature of NPC disease is the intracellular accumulation of cholesterol and gangliosides, predominantly in endolysosomes, mitochondrial cholesterol accumulation has also been reported. As accumulation of cholesterol in mitochondria is known to impair the transport of GSH into mitochondria, resulting in mitochondrial GSH (mGSH) depletion, we investigated the impact of mGSH recovery in NPC disease. We show that GSH ethyl ester (GSH-EE), but not N-acetylcysteine (NAC), restored the mGSH pool in liver and brain of Npc1
Indexed as
AcetylcysteineAnimalsCerebellumCholesterolGlutathioneHumansIntracellular Signaling Peptides and ProteinsLysosomesMiceMice, KnockoutMitochondriaMutationNiemann-Pick C1 ProteinNiemann-Pick Disease, Type COxidative PhosphorylationProteinsAcetylcysteineCholesterolGlutathioneIntracellular Signaling Peptides and ProteinsNiemann-Pick C1 ProteinNpc1 protein, mouseNpc2 protein, mouseProteinsVesicular Transport ProteinsCeramideCerebellumHepatosplenomegalyLysosomal disordersSphingolipids, Mitochondrial GSH
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