The role of lysosomal ion channels in lysosome dysfunction

Kendall, Rebekah L.; Holian, Andrij

doi:10.1080/08958378.2021.1876188

Cited by 18 publications

(11 citation statements)

References 115 publications

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“…This provides a model for using neural progenitor cells to transfer other foreign genes or factors to the CNS. Recent basic studies have shed light on the link between storage-related substances, lysosomal dysfunction, innate immune activation, and hyperinflammation that aggravate MPS symptoms, and these mechanisms could be important targets for new therapies (Kendall and Holian, 2021;Tillo et al, 2022;Xu and Núñez, 2023). Therefore, a new generation of ERT has been developed and investigated to overcome the problem that conventional ERT therapy does not reach the CNS, which we will talk about later.…”

Section: Discussionmentioning

confidence: 99%

The top 100 most cited articles on mucopolysaccharidoses: a bibliometric analysis

Liao,

Geng,

Yang

et al. 2024

Front. Genet.

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Background: Bibliometrics can trace general research trends in a particular field. Mucopolysaccharidoses (MPS), as a group of rare genetic diseases, seriously affect the quality of life of patients and their families. Scholars have devoted themselves to studying MPS’s pathogenesis and treatment modalities and have published many papers. Therefore, we conducted a bibliometric and visual study of the top 100 most highly cited articles to provide researchers with an indication of the current state of research and potential directions in the field.Methods: The Web of Science Core Collection was searched for articles on MPS from 1 January 1900, to 8 November 2023, and the top 100 most cited articles were screened. The title, year of publication, institution, country, and first author of the articles were extracted and statistically analyzed using Microsoft Excel 2007. Keyword co-occurrence and collaborative networks were analyzed using VOSviewer 1.6.16.Results: A total of 9,273 articles were retrieved, and the top 100 most cited articles were filtered out. The articles were cited 18,790 times, with an annual average of 188 citations (122–507). Forty-two journals published these articles, with Molecular Genetics and Metabolism and Proceedings of the National Academy of Sciences of the United States being the most published journal (N = 8), followed by Pediatrics (N = 7), Blood (N = 6). The United States (N = 68), the UK (N = 25), and Germany (N = 20) were the top contributing countries. The Royal Manchester Children’s Hospital (N = 20) and the University of North Carolina (N = 18) were the most contributing institutions. Muenzer J was the most prolific author (N = 14).Conclusion: We conducted a bibliometric and visual analysis of the top 100 cited articles in MPS. This study identifies the most influential articles currently available in the field of MPS, which provides a good basis for a better understanding of the disease and informs future research directions.

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Section: Discussionmentioning

confidence: 99%

The top 100 most cited articles on mucopolysaccharidoses: a bibliometric analysis

Liao,

Geng,

Yang

et al. 2024

Front. Genet.

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“…What is more, lysosomes are emerging as intracellular Ca 2+ stores (Zhong et al, 2017), and the activity of lysosomal ion channels and transporters maintains concentration gradients of K + , Ca 2+ , Na + , and Cl − across the lysosomal membrane. Once the lysosomal damage occurs, it would contribute to the Ca 2+ overloading and the disorder of lysosomal ion channel activity, which stimulates the activation of the NLRP3 inflammasome (Kendall and Holian, 2021). It has been proven that the restoration of lysosomal dysfunction could augment neuroprotection against ischemic stroke in neurons (Zhang et al, 2022).…”

Section: Lysosomal Damagementioning

confidence: 99%

NLRP3 Inflammasome Activation: A Therapeutic Target for Cerebral Ischemia–Reperfusion Injury

Wang

Ren

et al. 2022

Front. Mol. Neurosci.

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Millions of patients are suffering from ischemic stroke, it is urgent to figure out the pathogenesis of cerebral ischemia–reperfusion (I/R) injury in order to find an effective cure. After I/R injury, pro-inflammatory cytokines especially interleukin-1β (IL-1β) upregulates in ischemic brain cells, such as microglia and neuron. To ameliorate the inflammation after cerebral I/R injury, nucleotide-binding oligomerization domain (NOD), leucine-rich repeat (LRR), and pyrin domain-containing protein 3 (NLRP3) inflammasome is well-investigated. NLRP3 inflammasomes are complicated protein complexes that are activated by endogenous and exogenous danger signals to participate in the inflammatory response. The assembly and activation of the NLRP3 inflammasome lead to the caspase-1-dependent release of pro-inflammatory cytokines, such as interleukin (IL)-1β and IL-18. Furthermore, pyroptosis is a pro-inflammatory cell death that occurs in a dependent manner on NLRP3 inflammasomes after cerebral I/R injury. In this review, we summarized the assembly and activation of NLRP3 inflammasome; moreover, we also concluded the pivotal role of NLRP3 inflammasome and inhibitors, targeting the NLRP3 inflammasome in cerebral I/R injury.

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“…These have been implicated in various human pathological conditions associated with lysosomal storage diseases, such as neurodegenerative diseases, metabolic disorders and cancer (Ellison et al., 2023; Pfrieger, 2023; Shin et al., 2019; Toledano‐Zaragoza & Ledesma, 2020). Lysosomal storage diseases can arise from mutations in lysosomal enzymes, but naturally occurring variations in the expression and/or function of lysosomal ion channels and transporters can also lead to lysosomal storage diseases (Bissa et al., 2016; Cao et al., 2015; Feng et al., 2018; Huizing & Gahl, 2020; Kendall & Holian, 2021; Onyenwoke et al., 2015; Riederer et al., 2023; Shen et al., 2012; Zhang et al., 2018). Therefore, studying the electrophysiological properties of the lysosome and biophysics of ion transport systems in their native environment becomes crucial, because the establishment, maintenance and controlled changes in the membrane voltage are likely to play a fundamental physiological role.…”

Section: Introductionmentioning

confidence: 99%

“…Numerous ion transport systems are responsible for maintaining the electrical properties of the lysosomal membrane, and a failure to do so can have pathological consequences (Feng et al., 2018; Kendall & Holian, 2021; Riederer et al., 2023). At least 10 distinct ion transport systems have been identified in the lysosome, and these are responsible for the permeability to H + , K + , Na + , Ca 2+ and Cl − (Chen et al., 2017; Li et al., 2019; Riederer et al., 2023).…”

Section: Introductionmentioning

confidence: 99%

Optical recordings of organellar membrane potentials and the components of membrane conductance in lysosomes

Castillo‐Velasquez,

Matamala,

Becerra

et al. 2024

The Journal of Physiology

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The eukaryotic cell is highly compartmentalized with organelles. Owing to their function in transporting metabolites, metabolic intermediates and byproducts of metabolic activity, organelles are important players in the orchestration of cellular function. Recent advances in optical methods for interrogating the different aspects of organellar activity promise to revolutionize our ability to dissect cellular processes with unprecedented detail. The transport activity of organelles is usually coupled to the transport of charged species; therefore, it is not only associated with the metabolic landscape but also entangled with membrane potentials. In this context, the targeted expression of fluorescent probes for interrogating organellar membrane potential (Ψorg) emerges as a powerful approach, offering less‐invasive conditions and technical simplicity to interrogate cellular signalling and metabolism. Different research groups have made remarkable progress in adapting a variety of optical methods for measuring and monitoring Ψorg. These approaches include using potentiometric dyes, genetically encoded voltage indicators, hybrid fluorescence resonance energy transfer sensors and photoinduced electron transfer systems. These studies have provided consistent values for the resting potential of single‐membrane organelles, such as lysosomes, the Golgi and the endoplasmic reticulum. We can foresee the use of dynamic measurements of Ψorg to study fundamental problems in organellar physiology that are linked to serious cellular disorders. Here, we present an overview of the available techniques, a survey of the resting membrane potential of internal membranes and, finally, an open‐source mathematical model useful to interpret and interrogate membrane‐bound structures of small volume by using the lysosome as an example. image

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The role of lysosomal ion channels in lysosome dysfunction

Cited by 18 publications

References 115 publications

The top 100 most cited articles on mucopolysaccharidoses: a bibliometric analysis

The top 100 most cited articles on mucopolysaccharidoses: a bibliometric analysis

NLRP3 Inflammasome Activation: A Therapeutic Target for Cerebral Ischemia–Reperfusion Injury

Optical recordings of organellar membrane potentials and the components of membrane conductance in lysosomes

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