Endothelial glycocalyx degradation during sepsis: Causes and consequences
Matrix Biology Plus, 2021 The glycocalyx is a ubiquitous structure found on endothelial cells that extends into the vascular lumen. It is enriched in proteoglycans, which are proteins attached to the glycosaminoglycans heparan sulfate, chondroitin sulfate, dermatan sulfate ...
Ryan C. Sullivan +3 more
doaj +1 more source
Isolation and characterization of a heptaglycosylceramide from bovine erythrocyte membranes.
Journal of Lipid Research, 1979 A heptaglycosylceramide was isolated from bovine erythrocyte membranes. The structure was characterized to be Gal(alpha 1-3)Gal(beta 1-4)GlcNAc(beta1-3)Gal(beta 1-4)Glc-NAc(beta 1-4)al(beta 1-4)GlcCer.
J L Chien, S C Li, Y T Li
doaj +1 more source
Recommendations for the management of MPS IVA: systematic evidence- and consensus-based guidance. [PDF]
, 2019 IntroductionMucopolysaccharidosis (MPS) IVA or Morquio A syndrome is an autosomal recessive lysosomal storage disorder (LSD) caused by deficiency of the N-acetylgalactosamine-6-sulfatase (GALNS) enzyme, which impairs lysosomal degradation of keratan ...
Akyol, Mehmet Umut +30 more
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Strain-dependent porcine circovirus type 2 (PCV2) entry and replication in T-lymphoblasts [PDF]
, 2019 Porcine circovirus type 2 (PCV2) is the etiological agent of PCV2-associated diseases (PCVAD). PCV2 targets lymphoblasts, and pigs suffering from PCVAD display lymphocyte depletion in lymphoid tissues. PCV2 infection of lymphoblasts has not been studied.
Nauwynck, Hans +2 more
core +1 more source
Orthopedic management of the extremities in patients with Morquio A syndrome. [PDF]
, 2014 BackgroundMusculoskeletal involvement in Morquio A syndrome (mucopolysaccharidosis IVA; MPS IVA) contributes significantly to morbidity and mortality. While the spinal manifestations of the disorder have received considerable attention in the literature,
Bache, C Edward +6 more
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Establishment of Glycosaminoglycan Assays for Mucopolysaccharidoses
Metabolites, 2014 Mucopolysaccharidoses (MPS) are a group of lysosomal storage disorders caused by deficiency of the lysosomal enzymes essential for catabolism of glycosaminoglycans (GAGs).
Shunji Tomatsu +13 more
doaj +1 more source
Impact of elosulfase alfa in patients with morquio A syndrome who have limited ambulation: An open-label, phase 2 study. [PDF]
, 2017 Efficacy and safety of elosulfase alfa enzyme replacement therapy (ERT) were assessed in an open-label, phase 2, multi-national study in Morquio A patients aged ≥5 years unable to walk ≥30 meters in the 6-min walk test.
Berger, Kenneth I +11 more
core +1 more source
Proton conductivity of glycosaminoglycans.
PLoS ONE, 2019 Proton conductivity is important in many natural phenomena including oxidative phosphorylation in mitochondria and archaea, uncoupling membrane potentials by the antibiotic Gramicidin, and proton actuated bioluminescence in dinoflagellate.
John Selberg, Manping Jia, Marco Rolandi
doaj +1 more source
Expanding the Spectrum: A Rare Case of Morquio Syndrome With Bronchial Asthma and Seizure Disorder. [PDF]
Clin Case RepABSTRACT This case highlights a rare co‐existence of Morquio syndrome with bronchial asthma and myoclonic seizures, expanding the known clinical spectrum. It emphasizes the need for comprehensive, multidisciplinary management to address multisystem involvement and describes the challenges of treatment in resource‐limited settings where advanced ...
Bastola B +4 more
europepmc +2 more sources
Cornea organoids from human induced pluripotent stem cells. [PDF]
, 2017 The cornea is the transparent outermost surface of the eye, consisting of a stratified epithelium, a collagenous stroma and an innermost single-cell layered endothelium and providing 2/3 of the refractive power of the eye. Multiple diseases of the cornea
Adams, Sheila M +5 more
core +3 more sources