Lysosomal Storage Diseases: Heterogeneous Group of Disorders [PDF]
BioImpacts, 2013 The name of lysosomal storage diseases stems from the fact that in this category of disorders specific undegraded materials are stored in the lysosomes.
David A. Wenger +2 more
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LYSOSOMAL ENZYMES AND THEIR RECEPTORS
Annual Review of Biochemistry, 1986 PERSPECTIVES AND SUMMARY . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 167 BIOSYNTHESIS OF LYSOSOMAL ENZyMES. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 168 Synthesis and Modifications in Endoplasmic Reticulum. . .. . . . . . .
Figura, Kurt von, Hasilik, Andrej
openaire +4 more sources
Molecular Mechanisms in Lysosomal Storage Diseases: From Pathogenesis to Therapeutic Strategies
Biomedicines, 2022 Lysosomal storage diseases (LSDs) are a group of metabolic diseases caused by inborn mutations of lysosomal enzymes, which lead to lysosome substrate accumulation in various cell types [...]
Valeria De Pasquale +2 more
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Role of lysosomal enzymes released by alveolar macrophages in the pathogenesis of the acute phase of hypersensitivity pneumonitis [PDF]
Mediators of Inflammation, 1995 Hydrolytic enzymes are the major constituents of alveolar macrophages (AM) and have been shown to be involved in many aspects of the inflammatory pulmonary response.
J. L. Pérez-Arellano +5 more
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Lysosomal hydrolases, from waste-bags effectors to essential multipurpose enzymes in liver fibrosis [PDF]
Exploration of Digestive Diseases, 2023 Lysosomal hydrolases were once considered effectors of the waste disposal system of the cell, the endo-lysosomal system. However, they are now recognized as highly selective enzymes, which can modulate the function of several substrates, contributing to ...
Paloma Ruiz-Blázquez +2 more
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The glycosylation design space for recombinant lysosomal replacement enzymes produced in CHO cells [PDF]
Nature Communications, 2019 Lysosomal replacement enzymes are taken up by cell surface receptors that recognize glycans, the effects of different glycan features are unknown. Here the authors present a gene engineering screen in CHO cells that allows custom N-glycan-decorated ...
Weihua Tian +17 more
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Evaluation of artificial signal peptides for secretion of two lysosomal enzymes in CHO cells. [PDF]
Biochem J, 2021 Enzyme replacement therapy (ERT) is a scientifically rational and clinically proven treatment for lysosomal storage diseases. Most enzymes used for ERT are purified from the culture supernatant of mammalian cells.
Cheng KW +6 more
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Streptozotocin-induced diabetes mellitus affects lysosomal enzymes in rat liver
Brazilian Journal of Medical and Biological Research, 2014 It has been previously shown that dextran sulfate administered to diabetic rats accumulates in the liver and kidney, and this could be due to a malfunction of the lysosomal digestive pathway.
G.B. Peres +3 more
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A CLN6-CLN8 complex recruits lysosomal enzymes at the ER for Golgi transfer. [PDF]
J Clin Invest, 2020 Lysosomal enzymes are synthesized in the endoplasmic reticulum (ER) and transferred to the Golgi complex by interaction with the Batten disease protein CLN8 (ceroid lipofuscinosis, neuronal, 8).
Bajaj L +15 more
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Brazilian Journal of Pharmaceutical Sciences, 2022 Gout is a form of metabolic arthritis originated on grounds of increased accumulation of monosodium urate (MSU) crystals in joints. Current study focuses on anti-arthritic activities of β-carotene on MSU crystal-induced gouty arthritis rats in comparison
Lavinya B Udhaya +5 more
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