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Lysosomal Biogenesis in Lysosomal Storage Disorders
Experimental Cell Research, 1997Lysosomal biogenesis is an orchestration of the structural and functional elements of the lysosome to form an integrated organelle and involves the synthesis, targeting, functional residence, and turnover of the proteins that comprise the lysosome. We have investigated lysosomal biogenesis during the formation and dissipation of storage vacuoles in two
Karageorgos, L. +6 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).
Jacob Valk, Marjo S. van der Knaap
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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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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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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.
Michel Jadot +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.
Michel Jadot +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.
Andrej Hasilik +2 more
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The role of lysosomal ion channels in lysosome dysfunction
Inhalation Toxicology, 2021Lysosomes offer a unique arrangement of degradative, exocytic, and signaling capabilities that make their continued function critical to cellular homeostasis. Lysosomes owe their function to the activity of lysosomal ion channels and transporters, which maintain concentration gradients of H+, K+, Ca2+, Na+, and Cl- across the lysosomal membrane.
Rebekah L. Kendall, Andrij Holian
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PROTEOMICS, 2010
AbstractLysosomes are organelles of eukaryotic cells that are critically involved in the degradation of macromolecules mainly delivered by endocytosis and autophagocytosis. Degradation is achieved by more than 60 hydrolases sequestered by a single phospholipid bilayer.
Bernd Schröder +3 more
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AbstractLysosomes are organelles of eukaryotic cells that are critically involved in the degradation of macromolecules mainly delivered by endocytosis and autophagocytosis. Degradation is achieved by more than 60 hydrolases sequestered by a single phospholipid bilayer.
Bernd Schröder +3 more
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Annual Review of Cell Biology, 1989
Article de synthese sur les lysosomes: biogenese, definition, role biologique. Les enzymes lysosomales sont decrites ainsi que leur role dans l'exo et l'endocytose.
Ira Mellman, Stuart Kornfeld
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Article de synthese sur les lysosomes: biogenese, definition, role biologique. Les enzymes lysosomales sont decrites ainsi que leur role dans l'exo et l'endocytose.
Ira Mellman, Stuart Kornfeld
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The Cytoplasm: Lysosomes and Lysosomal Disorders
2015Lysosomes are enriched in hydrolytic enzymes working at low pH, which include proteases, lipases, glycosidases, nucleases, phosphatases, and sulfatases, and are responsible for elimination of “unwanted” molecules derived from both the cell itself (autophagy, cf. Fig. 74) or from outside of the cells (heterophagy). However, contrary to the historic view
Jürgen Roth, Margit Pavelka
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