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The Journal of Pediatrics, 1966
The cells of most vertebrate tissues possess specialized structures called lysosomes.These organelles contain enzymes which can digest proteins, carbohydrates, and other cell constituents. Lysosomes appear to play a vital role in the life and death of cells. They have been linked to important physiological processes and to the pathogenesis of a variety
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The cells of most vertebrate tissues possess specialized structures called lysosomes.These organelles contain enzymes which can digest proteins, carbohydrates, and other cell constituents. Lysosomes appear to play a vital role in the life and death of cells. They have been linked to important physiological processes and to the pathogenesis of a variety
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2010
Abstract The lysosome is a ubiquitous, single membrane-bond intracellular organelle which continuously recycles biological macromolecules: it not only breaks down cell components but has a dynamic role in nutrient and energy sensing that, through regulatory signalling, is critical for homeostasis and metabolic economy of the cell ...
P.B. Deegan, T.M. Cox
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Abstract The lysosome is a ubiquitous, single membrane-bond intracellular organelle which continuously recycles biological macromolecules: it not only breaks down cell components but has a dynamic role in nutrient and energy sensing that, through regulatory signalling, is critical for homeostasis and metabolic economy of the cell ...
P.B. Deegan, T.M. Cox
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Pathogenic mechanisms in lysosomal disease: a reappraisal of the role of the lysosome
Acta Paediatrica, 2007AbstractThe view that lysosomes simply represent end organelles in the serial degradation of polymeric molecules derived from the cell surface and its interior has led to major misconceptions about the nature of lysosomal storage diseases and the pathogenic cascades that characterize them.
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The Role of Lysosomes in Inflammation and Disease
Annual Review of Medicine, 1967Therefore, this review will limit it self to three aspects of this rapidly enlarging field: (a) Iysosomes in tissue injury and inflammation; (b) Iysosomes in immunopathology; and (c) Iyso somes in human disease. Lysosomes comprise a heterogeneous group of cyt oplasmic organelles, the ultrastructural appearance of which in tissues depends upon the ...
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[Lysosomes and lysosomal storage diseases].
Journal de la Societe de biologie, 2002Lysosomal storage disorders (LSDs) are monogenic inborn errors of metabolism. Various groups have been delineated according to the affected pathway and the accumulated substrate, and new entities are still being identified. They are severe disorders with a heterogeneous clinical spectrum encompassing visceral, skeletal and neurologic involvement, and ...
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Lysosomal diseases: diagnostic update
Journal of Inherited Metabolic Disease, 2014AbstractTechnological developments in newborn and population screening, biomarker discovery for monitoring treatment and rapid high throughput DNA sequencing are having a great impact on the diagnostic procedure for symptomatic patients with lysosomal storage diseases.
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Innovative Treatments for Lysosomal Diseases
Best Practice & Research Clinical Endocrinology & Metabolism, 2015Striking therapeutic advances for lysosomal diseases have harnessed the biology of this organelle and illustrate its central rôle in the dynamic economy of the cell. Further Innovation will require improved protein-targetting or realization of therapeutic gene- and cell transfer stratagems.
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La Revue du praticien, 1994
Lysosomal diseases are severe genetic enzymopathies generally affecting the child, with a progressively fatal issue in the first few years of life. The group is very heterogeneous regarding: the number of included affections (about 30), as well as the clinical, biological and molecular manifestations.
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Lysosomal diseases are severe genetic enzymopathies generally affecting the child, with a progressively fatal issue in the first few years of life. The group is very heterogeneous regarding: the number of included affections (about 30), as well as the clinical, biological and molecular manifestations.
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