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Lysosomal Biogenesis in Lysosomal Storage Disorders

Experimental Cell Research, 1997
Lysosomal 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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Lysosomal proteinases

Acta Histochemica, 1987
A characteristic of lysosomal cysteine proteinases is given by their kinetic constants with specific substrates, their sequence homology, and their reactivity with monospecific polyclonal antibodies.
H, Kirschke, B, Wiederanders
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Lysosomes, Lysosomal Enzymes, and Cancer

1992
Publisher Summary Lysosomes are membrane-enclosed cytoplasmic organelles, which posses an acidic interior that contains many hydrolytic enzymes. Their major function is in the the degradation of macromolecules, which may be cellular or foreign in origin.
M J, Boyer, I F, Tannock
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Angulate Lysosomes

Ultrastructural Pathology, 1983
Under many circumstances, macrophages accumulate lipids (possibly in combination with other materials) in the form of micelles that by their rigidity and size impart an irregular, angulate shape to the lysosomes in which they are stored. When macrophages contain large numbers of these angulate lysosomes, they have a characteristic light microscopic ...
K P, Dingemans   +2 more
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Secretory lysosomes

Nature Reviews Molecular Cell Biology, 2002
Regulated secretion of stored secretory products is important in many cell types. In contrast to professional secretory cells, which store their secretory products in specialized secretory granules, some secretory cells store their secretory proteins in a dual-function organelle, called a secretory lysosome.
Emma J, Blott, Gillian M, Griffiths
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Rethinking lysosomes and lysosomal disease

Neuroscience Letters, 2021
Lysosomal 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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Lysosome electrophysiology

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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