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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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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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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 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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PROTEOMICS, 2010
Abstract Lysosomes 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 A, Schröder +3 more
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Abstract Lysosomes 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 A, Schröder +3 more
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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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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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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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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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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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1967
Publisher Summary This chapter focuses on the histochemistry of lysosomes. Lysosomes are defined originally as a biochemical entity. Consequently, the biochemical criteria form a basis for the histochemical identification of these particles. Ideally, the prime criteria which appear necessary for the demonstration of a lysosome are (1) the presence of
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Publisher Summary This chapter focuses on the histochemistry of lysosomes. Lysosomes are defined originally as a biochemical entity. Consequently, the biochemical criteria form a basis for the histochemical identification of these particles. Ideally, the prime criteria which appear necessary for the demonstration of a lysosome are (1) the presence of
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Nature, 1966
IN recent years there has been much interest in the role of crystals in causing acute attacks of gout. Faires and his colleagues at Philadelphia1 and Seegmiller et al. at Bethesda2 were able to produce such attacks by injecting microcrystalline sodium monourate into gouty and non-gouty volunteers.
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IN recent years there has been much interest in the role of crystals in causing acute attacks of gout. Faires and his colleagues at Philadelphia1 and Seegmiller et al. at Bethesda2 were able to produce such attacks by injecting microcrystalline sodium monourate into gouty and non-gouty volunteers.
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