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Rethinking lysosomes and lysosomal disease
Neuroscience Letters, 2021Lysosomal storage diseases were recognized and defined over a century ago as a class of disorders affecting mostly children and causing systemic disease often accompanied by major neurological consequences. Since their discovery, research focused on understanding their causes has been an important driver of our ever-expanding knowledge of cell biology ...
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2015
The physiology and functions of ion channels have been major topics of interest in biomedical research. Patch clamping is one of the most powerful techniques used in the study of ion channels and has been widely applied to the investigation of electrical properties of ion channels on the plasma membrane in a variety of cells.
Xi Z, Zhong, Xian-Ping, Dong
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The physiology and functions of ion channels have been major topics of interest in biomedical research. Patch clamping is one of the most powerful techniques used in the study of ion channels and has been widely applied to the investigation of electrical properties of ion channels on the plasma membrane in a variety of cells.
Xi Z, Zhong, Xian-Ping, Dong
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Lysosomal Ion Channels and Lysosome–Organelle Interactions
2023Intracellular organelles exchange their luminal contents with each other via both vesicular and non-vesicular mechanisms. By forming membrane contact sites (MCSs) with ER and mitochondria, lysosomes mediate bidirectional transport of metabolites and ions between lysosomes and organelles that regulate lysosomal physiology, movement, membrane remodeling,
Weijie, Cai +3 more
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Lysosomes and Lysosomal Disorders
1989Lysosomes are membranous vesicles that are present in almost all kinds of body cells. Their number varies greatly from one cell to another, depending on its type and function. They have been given their name because they are small bodies (soma = body) containing various enzymes that are hydrolytic (lysis = dissolution).
Marjo S. van der Knaap, Jacob Valk
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Paediatrics and Child Health, 2002
Although most lysosomal storage disorders present in infancy or early childhood with a progressive condition often associated with dysmorphism, considerable genetic heterogeneity exists resulting in a range of illnesses that can include a dramatic neonatal presentation.
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Although most lysosomal storage disorders present in infancy or early childhood with a progressive condition often associated with dysmorphism, considerable genetic heterogeneity exists resulting in a range of illnesses that can include a dramatic neonatal presentation.
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Current Drug Metabolism, 2012
Lysosomes are cytoplasmic organelles delimited by a single membrane and filled with a variety of hydrolytic enzymes active at acidic pH and collectively capable to degrade the vast majority of macromolecules entering lysosomes via endocytosis, phagocytosis or autophagy.
Hamer, Isabelle +3 more
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Lysosomes are cytoplasmic organelles delimited by a single membrane and filled with a variety of hydrolytic enzymes active at acidic pH and collectively capable to degrade the vast majority of macromolecules entering lysosomes via endocytosis, phagocytosis or autophagy.
Hamer, Isabelle +3 more
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Lysosomal Membrane Proteomics and Biogenesis of Lysosomes
Molecular Neurobiology, 2005This review focuses on events involved in the biogenesis of the lysosome. This organelle contains a diverse array of soluble, luminal proteins capable of digesting all the macromolecules in the cell. Altered function of lysosomes or its constituent enzymes has been implicated in a host of human pathologies, including storage diseases, cancer, and ...
Richard D, Bagshaw +2 more
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Lysosomes and lysosomal cathepsins in cell death
Biochimica et Biophysica Acta (BBA) - Proteins and Proteomics, 2012Lysosomes are the key degradative compartments of the cell. Lysosomal cathepsins, which are enclosed in the lysosomes, help to maintain the homeostasis of the cell's metabolism by participating in the degradation of heterophagic and autophagic material. Following the targeted lysosomal membrane's destabilization, the cathepsins can be released into the
Repnik, Urška +3 more
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Intracellular trafficking of lysosomal proteins and lysosomes
Int. Journal of Clinical Pharmacology and Therapeutics, 2009In the synthesis and trafficking of precursors of most lysosomal matrix proteins, the stages necessary for lysosomal delivery include the addition of phosphorylated mannose-rich oligosaccharides, binding of the modified proteins to receptors, their segregation from the secretory pathways and delivery to the endosomal pathway.
A, Hasilik, C, Wrocklage, B, Schröder
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2022
Lysosomal storage diseases (LSDs) are a group of inherited metabolic disorders that result from lysosomal dysfunction. Most LSDs are autosomal recessively inherited disorders, with only three exceptions that are X-linked. The overall incidence is about 1 in 5000 live births.
Funghini, Silvia +3 more
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Lysosomal storage diseases (LSDs) are a group of inherited metabolic disorders that result from lysosomal dysfunction. Most LSDs are autosomal recessively inherited disorders, with only three exceptions that are X-linked. The overall incidence is about 1 in 5000 live births.
Funghini, Silvia +3 more
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