The rapidly evolving view of lysosomal storage diseases
Lysosomal storage diseases are a group of metabolic disorders caused by deficiencies of
several components of lysosomal function. Most commonly affected are lysosomal …
several components of lysosomal function. Most commonly affected are lysosomal …
Mucopolysaccharidosis type II: one hundred years of research, diagnosis, and treatment
Mucopolysaccharidosis type II (MPS II, Hunter syndrome) was first described by Dr. Charles
Hunter in 1917. Since then, about one hundred years have passed and Hunter syndrome …
Hunter in 1917. Since then, about one hundred years have passed and Hunter syndrome …
Brain delivery and activity of a lysosomal enzyme using a blood-brain barrier transport vehicle in mice
JC Ullman, A Arguello, JA Getz, A Bhalla… - Science Translational …, 2020 - science.org
Most lysosomal storage diseases (LSDs) involve progressive central nervous system (CNS)
impairment, resulting from deficiency of a lysosomal enzyme. Treatment of neuronopathic …
impairment, resulting from deficiency of a lysosomal enzyme. Treatment of neuronopathic …
Autophagy–lysosomal-associated neuronal death in neurodegenerative disease
RA Nixon - Acta Neuropathologica, 2024 - Springer
Autophagy, the major lysosomal pathway for degrading damaged or obsolete constituents,
protects neurons by eliminating toxic organelles and peptides, restoring nutrient and energy …
protects neurons by eliminating toxic organelles and peptides, restoring nutrient and energy …
[HTML][HTML] Heparan sulfate proteoglycans: The sweet side of development turns sour in mucopolysaccharidoses
Heparan sulfate proteoglycans (HSPGs) are complex carbohydrate-modified proteins
ubiquitously expressed on cell surfaces, extracellular matrix and basement membrane of …
ubiquitously expressed on cell surfaces, extracellular matrix and basement membrane of …
Innate immunity in mucopolysaccharide diseases
O Mandolfo, H Parker, B Bigger - International Journal of Molecular …, 2022 - mdpi.com
Mucopolysaccharidoses are rare paediatric lysosomal storage disorders, characterised by
accumulation of glycosaminoglycans within lysosomes. This is caused by deficiencies in …
accumulation of glycosaminoglycans within lysosomes. This is caused by deficiencies in …
Differences in MPS I and MPS II disease manifestations
Mucopolysaccharidosis (MPS) type I and II are two closely related lysosomal storage
diseases associated with disrupted glycosaminoglycan catabolism. In MPS II, the first step of …
diseases associated with disrupted glycosaminoglycan catabolism. In MPS II, the first step of …
Targeting brain disease in MPSII: preclinical evaluation of IDS-loaded PLGA nanoparticles
L Rigon, M Salvalaio, F Pederzoli, E Legnini… - International journal of …, 2019 - mdpi.com
Mucopolysaccharidosis type II (MPSII) is a lysosomal storage disorder due to the deficit of
the enzyme iduronate 2-sulfatase (IDS), which leads to the accumulation of …
the enzyme iduronate 2-sulfatase (IDS), which leads to the accumulation of …
Cathepsins in the pathophysiology of mucopolysaccharidoses: new perspectives for therapy
Cathepsins (CTSs) are ubiquitously expressed proteases normally found in the
endolysosomal compartment where they mediate protein degradation and turnover …
endolysosomal compartment where they mediate protein degradation and turnover …
Network Pharmacology-Based Strategy for Elucidating the Molecular Basis Forthe Pharmacologic Effects of Licorice (Glycyrrhiza spp.)
J Chen, LF Li, XR Hu, F Wei, S Ma - Frontiers in Pharmacology, 2021 - frontiersin.org
Licorice (Glycyrrhiza spp.) is used widely in traditional Chinese medicine (TCM) due to its
numerous pharmacologic effects. However, the mechanisms of action of the chemical …
numerous pharmacologic effects. However, the mechanisms of action of the chemical …