IUBMB Life (International Union of Biochemistry and Molecular Biology: Life), 2006 The lysosomal compartment is the place for cellular degradation of endocytosed and autophagocytosed material and a center for normal turnover of organelles as well as most long-lived proteins. Lysosomes were long considered stable structures that broke and released their many hydrolytic enzymes only following necrotic cell death.
A, Terman +3 more
openaire +2 more sources
Orphanet Journal of Rare Diseases, 2018 Background Rare diseases are often un- or misdiagnosed for extended periods, resulting in a long diagnostic delay that may significantly add to the burden of the disease.
Gé-Ann Kuiper +3 more
doaj +1 more source
Primary fibroblasts from CSPα mutation carriers recapitulate hallmarks of the adult onset neuronal ceroid lipofuscinosis [PDF]
, 2017 Mutations in the co- chaperone protein, CSPα, cause an autosomal dominant, adult-neuronal ceroid lipofuscinosis (AD-ANCL). The current understanding of CSPα function exclusively at the synapse fails to explain the autophagy-lysosome pathway (ALP ...
Benitez, Bruno A, Sands, Mark S
core +2 more sources
Simvastatin improves lysosome function via enhancing lysosome biogenesis in endothelial cells
Frontiers in Bioscience-Landmark, 2020 Nlrp3 inflammasomes were shown to play a critical role in triggering obesity-associated early onsets of cardiovascular complications such as endothelial barrier dysfunction with endothelial hyperpermeability.
Youzhi Zhang +5 more
doaj +1 more source
Phagosome-lysosome fusion is a calcium-independent event in macrophages. [PDF]
, 1996 Phagosome-lysosome membrane fusion is a highly regulated event that is essential for intracellular killing of microorganisms. Functionally, it represents a form of polarized regulated secretion, which is classically dependent on increases in ...
Ernst, JD +5 more
core +2 more sources
Neurophysiological, behavioral and morphological abnormalities in the Fabry knockout mice
Neurobiology of Disease, 2009 Fabry disease (OMIM 301500) is a rare X-linked recessive disorder caused by mutations in the α-galactosidase gene (GLA). Loss of α-galactosidase (α-Gal) activity leads to the abnormal accumulation of glycosphingolipids in lysosomes predominantly of ...
L.G. Rodrigues +7 more
doaj +1 more source
VCP-dependent muscle degeneration is linked to defects in a dynamic tubular lysosomal network in vivo. [PDF]
, 2015 Lysosomes are classically viewed as vesicular structures to which cargos are delivered for degradation. Here, we identify a network of dynamic, tubular lysosomes that extends throughout Drosophila muscle, in vivo. Live imaging reveals that autophagosomes
Davis, Graeme W +4 more
core +3 more sources
Lysosome assembly and disassembly changes endocytosis rate through the Leishmania cell cycle
MicrobiologyOpen, 2020 The Leishmania lysosome has an atypical structure, consisting of an elongated vesicle‐filled tubule running along the anterior–posterior axis of the cell, which is termed the multivesicular tubule (MVT) lysosome.
Ziyin Wang +2 more
doaj +1 more source
Alginate oligosaccharides can maintain activities of lysosomes under low pH condition
Scientific Reports, 2021 The objective of this study was to report that lysosome extracted from egg white could be used as a drug through oral administration for treating diseases by using pH sensitive alginate oligosaccharides.
Ra-Mi Park +4 more
doaj +1 more source
A proteolytic cascade controls lysosome rupture and necrotic cell death mediated by lysosome-destabilizing adjuvants. [PDF]
PLoS ONE, 2014 Recent studies have linked necrotic cell death and proteolysis of inflammatory proteins to the adaptive immune response mediated by the lysosome-destabilizing adjuvants, alum and Leu-Leu-OMe (LLOMe). However, the mechanism by which lysosome-destabilizing
Jürgen Brojatsch +6 more
doaj +1 more source