Influence of length and conformation of saccharide head groups on the mechanics of glycolipid membranes: Unraveled by off-specular neutron scattering

Yamamoto, Akihisa; Abuillan, Wasim; Burk, Alexandra; Körner, Alexander; Ries, Annika; Werz, Daniel B.; Demé, Bruno; Tanaka, Motomu

doi:10.1063/1.4918585

Cited by 6 publications

(4 citation statements)

References 44 publications

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“…X-ray reflectivity (XRR) and neutron reflectivity (NR) have been applied to discern the equilibrium structure of various organic thin films, including glassy polymers, block copolymer monolayers at the air/water interface, − lipopolysaccharide monolayers supported on planar hydrophobic substrates and/or the air/water interface, − layer-by-layer multilayered films, , and polymer brushes produced by grafting-to or grafting-from approaches on solid substrates. ,,,− In particular, NR is a powerful tool for shedding light on the swollen polymer brush structure in contact with liquids. NR provides quantitative insights into the polymer component volume fraction profile normal to the substrate from the scattering length density (SLD) profile.…”

Section: Introductionmentioning

confidence: 99%

Hydration State Variation of Polyzwitterion Brushes through Interplay with Ions

Higaki

Kobayashi

Takahara³

2020

Langmuir

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Polyzwitterions have emerged as a new class of antifouling materials alternating poly(ethylene glycol). The exemplary biopassivation and lubrication behaviors are often attributed to the particular chemical structure of zwitterions, which involve a large dipole moment of the charged groups and a neutral net charge, while the hydration state and dynamics also associate with these characteristics. Polymer brushes composed of surface-tethered polyzwitterion chains produced by surface-initiated controlled radical polymerization have been developed as thin films which exhibit excellent antifouling and lubrication properties. In past decades, numerous studies have been devoted to examining the structure and dynamics of polyzwitterion brush chains in aqueous solutions. This feature article provides an overview of recent studies exploring the hydration state of polyzwitterion brushes with specular neutron reflectivity, highlights some newly published work on the nonuniform equilibrium structure, ion concentration dependence, ion specificity, and the effects of charge spacer length in the zwitterions, and discusses future perspective in this field.

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Section: Introductionmentioning

confidence: 99%

Hydration State Variation of Polyzwitterion Brushes through Interplay with Ions

Higaki

Kobayashi

Takahara³

2020

Langmuir

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“…Increase in expression of Gb3 in the plasma membrane of AGA gene-silenced endothelial cells has been documented [26]. Increased amounts of Gb3 in glycolipid membranes affect the mechanical properties of the membrane by increasing bending stiffness [38]. Changes in the mechanical properties of cellular membranes could potentially influence vesicle formation and fusion.…”

Section: Discussionmentioning

confidence: 99%

Development of a model system for neuronal dysfunction in Fabry disease

Kaneski

Brady

Hanover

et al. 2016

Molecular Genetics and Metabolism

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Fabry disease is a glycosphingolipid storage disorder that is caused by a genetic deficiency of the enzyme alpha-galactosidase A (AGA, EC 3.2.1.22). It is a multisystem disease that affects the vascular, cardiac, renal, and nervous systems. One of the hallmarks of this disorder is neuropathic pain and sympathetic and parasympathetic nervous dysfunction. The exact mechanism by which changes in AGA activity result in change in neuronal function is not clear, partly due to of a lack of relevant model systems. In this study, we report the development of an in vitro model system to study neuronal dysfunction in Fabry disease by using short-hairpin RNA to create a stable knock-down of AGA in the human cholinergic neuronal cell line, LA-N-2. We show that gene-silenced cells show specifically reduced AGA activity and store globotriaosylceramide. In gene-silenced cells, release of the neurotransmitter acetylcholine is significantly reduced, demonstrating that this model may be used to study specific neuronal functions such as neurotransmitter release in Fabry disease.

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“…Furthermore, in other lysosomal storage disorders, such as Gaucher disease, some authors reported how glucosyl sphingosine (glucopsychosine), an analogue of lyso-Gb3, has a toxic effect on cultured neuronal cells [ 34 ]. Patients with Anderson–Fabry disease (AFD) are susceptible to cerebrovascular disease, and this condition is observed more frequently in young individuals [ 34 , 35 , 36 ]. They may experience ischemic strokes at a higher rate compared to the general population of similar age groups.…”

Section: Molecular Pathogenesis Of Central Nervous System Involvement...mentioning

confidence: 99%

Molecular Pathogenesis of Central and Peripheral Nervous System Complications in Anderson–Fabry Disease

Tuttolomondo,

Baglio,

Riolo

et al. 2023

IJMS

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Fabry disease (FD) is a recessive monogenic disease linked to chromosome X due to more than two hundred mutations in the alfa-galactosidase A (GLA) gene. Modifications of the GLA gene may cause the progressive accumulation of globotriaosylceramide (Gb3) and its deacylated form, globotriasylsphingosine (lyso-Gb3), in lysosomes of several types of cells of the heart, kidneys, skin, eyes, peripheral and central nervous system (not clearly and fully demonstrated), and gut with different and pleiotropic clinical symptoms. Among the main symptoms are acroparesthesias and pain crisis (involving the peripheral nervous system), hypohidrosis, abdominal pain, gut motility abnormalities (involving the autonomic system), and finally, cerebrovascular ischemic events due to macrovascular involvement (TIA and stroke) and lacunar strokes and white matter abnormalities due to a small vessel disease (SVS). Gb3 lysosomal accumulation causes cytoplasmatic disruption and subsequent cell death. Additional consequences of Gb3 deposits are inflammatory processes, abnormalities of leukocyte function, and impaired trafficking of some types of immune cells, including lymphocytes, monocytes, CD8+ cells, B cells, and dendritic cells. The involvement of inflammation in AFD pathogenesis conflicts with the reported poor correlation between CRP levels as an inflammation marker and clinical scores such as the Mainz Severity Score Index (MSSI). Also, some authors have suggested an autoimmune reaction is involved in the disease’s pathogenetic mechanism after the α-galactosidase A deficiency. Some studies have reported a high degree of neuronal apoptosis inhibiting protein as a critical anti-apoptotic mediator in children with Fabry disease compared to healthy controls. Notably, this apoptotic upregulation did not change after treatment with enzymatic replacement therapy (ERT), with a further upregulation of the apoptosis-inducing factor after ERT started. Gb3-accumulation has been reported to increase the degree of oxidative stress indexes and the production of reactive oxygen species (ROS). Lipids and proteins have been reported as oxidized and not functioning. Thus, neurological complications are linked to different pathogenetic molecular mechanisms. Progressive accumulation of Gb3 represents a possible pathogenetic event of peripheral nerve involvement. In contrast, central nervous system participation in the clinical setting of cerebrovascular ischemic events seems to be due to the epitheliopathy of Anderson–Fabry disease with lacunar lesions and white matter hyperintensities (WMHs). In this review manuscript, we revised molecular mechanisms of peripheral and central neurological complications of Anderson–Fabry Disease. The management of Fabry disease may be improved by the identification of biomarkers that reflect the clinical course, severity, and progression of the disease. Intensive research on biomarkers has been conducted over the years to detect novel markers that may potentially be used in clinical practice as a screening tool, in the context of the diagnostic process and as an indicator of response to treatment. Recent proteomic or metabolomic studies are in progress, investigating plasma proteome profiles in Fabry patients: these assessments may be useful to characterize the molecular pathology of the disease, improve the diagnostic process, and monitor the response to treatment.

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Influence of length and conformation of saccharide head groups on the mechanics of glycolipid membranes: Unraveled by off-specular neutron scattering

Cited by 6 publications

References 44 publications

Hydration State Variation of Polyzwitterion Brushes through Interplay with Ions

Hydration State Variation of Polyzwitterion Brushes through Interplay with Ions

Development of a model system for neuronal dysfunction in Fabry disease

Molecular Pathogenesis of Central and Peripheral Nervous System Complications in Anderson–Fabry Disease

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