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https://www.readbyqxmd.com/read/27922006/structural-basis-of-myelin-associated-glycoprotein-adhesion-and-signalling
#1
Matti F Pronker, Suzanne Lemstra, Joost Snijder, Albert J R Heck, Dominique M E Thies-Weesie, R Jeroen Pasterkamp, Bert J C Janssen
Myelin-associated glycoprotein (MAG) is a myelin-expressed cell-adhesion and bi-directional signalling molecule. MAG maintains the myelin-axon spacing by interacting with specific neuronal glycolipids (gangliosides), inhibits axon regeneration and controls myelin formation. The mechanisms underlying MAG adhesion and signalling are unresolved. We present crystal structures of the MAG full ectodomain, which reveal an extended conformation of five Ig domains and a homodimeric arrangement involving membrane-proximal domains Ig4 and Ig5...
December 6, 2016: Nature Communications
https://www.readbyqxmd.com/read/27921176/two-feet-on-the-membrane-uptake-of-clostridial-neurotoxins
#2
Andreas Rummel
The extraordinary potency of botulinum neurotoxins (BoNT) and tetanus neurotoxin (TeNT) is mediated by their high neurospecificity, targeting peripheral cholinergic motoneurons leading to flaccid and spastic paralysis, respectively, and successive respiratory failure. Complex polysialo gangliosides accumulate BoNT and TeNT on the plasma membrane. The ganglioside binding in BoNT/A, B, E, F, G, and TeNT occurs via a conserved ganglioside-binding pocket within the most carboxyl-terminal 25 kDa domain HCC, whereas BoNT/C, DC, and D display here two different ganglioside binding sites...
December 1, 2016: Current Topics in Microbiology and Immunology
https://www.readbyqxmd.com/read/27916751/-autoantibodies-in-chronic-inflammatory-neuropathies
#3
Ryo Yamasaki
Autoantibodies in chronic demyelinating neuropathies have been explored for several years. Recently, the peptides in the nodes of Ranvier have been the focus of attention in finding targets of autoantibodies. Until now, the most popular autoantibodies have been contactin-1 and neurofascin-155 for chronic demyelinating polyradiculoneuropathy (CIDP), GM1-ganglioside for multifocal motor neuropathy, and myelin-associated glycoprotein for polyneuropathy associated with monoclonal gammopathy of unknown significance...
December 2016: Brain and Nerve, Shinkei Kenkyū No Shinpo
https://www.readbyqxmd.com/read/27916750/-pathophysiology-of-ataxia-in-fisher-syndrome
#4
Satoshi Kuwabara
Fisher syndrome is regarded as a peculiar inflammatory neuropathy associated with ophthalmoplegia, ataxia, and areflexia. The disorder is associated with preceding infection, cerebrospinal fluid albumino-cytological dissociation, and spontaneous recovery, and regarded as a variant of Guillain-Barré syndrome. The discovery of anti-GQ1b IgG antibodies led to dramatic advances in understanding the pathophysiology of Fisher syndrome. The lesions in Fisher syndrome are determined by expression of ganglioside GQ1b in the human nervous system...
December 2016: Brain and Nerve, Shinkei Kenkyū No Shinpo
https://www.readbyqxmd.com/read/27911407/preparation-of-cd4-t-cells-for-analysis-of-gd3-and-gd2-ganglioside-membrane-expression-by-microscopy
#5
Tania M Villanueva-Cabello, Iván Martinez-Duncker
The methods described herein for activation of naïve CD4(+) T cells in suspension and their adherence in coverslips for confocal microscopy analysis allow the spatial localization and visualization of gangliosides involved in CD4(+) T cell activation, that complement expression profiling experiments such as flow cytometry, western blotting or real-time PCR. The quantification of ganglioside expression through flow cytometry and their cellular localization through microscopy can be obtained by the use of anti-ganglioside antibodies with high affinity and specificity...
November 8, 2016: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/27909553/effects-of-gangliosides-from-deer-bone-extract-on-the-gene-expressions-of-matrix-metalloproteinases-and-collagen-type-ii-in-interleukin-1%C3%AE-induced-osteoarthritic-chondrocytes
#6
Hyung Joo Suh, Hyunji Lee, Byung Jung Min, Sung Ug Jung, Eun Young Jung
BACKGROUND/OBJECTIVES: We investigated the anti-osteoarthritic effects of deer bone extract on the gene expressions of matrix metalloproteinases (MMPs) and collagen type II (COL2) in interleukin-1β-induced osteoarthritis (OA) chondrocytes. MATERIALS/METHODS: Primary rabbit chondrocytes were treated as follows: CON (PBS treatment), NC (IL-1β treatment), PC (IL-1β + 100 µg/mL glucosamine sulphate/chondroitin sulphate mixture), and DB (IL-1β + 100 µg/mL deer bone extract)...
December 2016: Nutrition Research and Practice
https://www.readbyqxmd.com/read/27908207/anti-sulfatide-reactivity-in-patients-with-celiac-disease
#7
Domenica Saccomanno, Carolina Tomba, Francesca Magri, Philippe Backelandt, Leda Roncoroni, Luisa Doneda, Maria Teresa Bardella, Giacomo Pietro Comi, Nereo Bresolin, Dario Conte, Luca Elli
OBJECTIVE: To explore a possible significance of the presence of anti-ganglioside and anti-sulfatide antibodies in sera of adult patients with celiac disease (CD) in different clinical scenario. METHODS: We selected 22 adult patients with newly diagnosed CD and 20 age-sex matched non-CD controls. Patients' serum was tested - before and after at least 6 months on a gluten-free diet (GFD) - for anti-GM1, GM2, GM3, GD1a, GD1b, GD3, GT1a, GT1b, GQ1b and sulfatide IgM, IgG and IgA auto-antibodies, by means of a dot blot technique and enzyme-linked immunosorbent assay (ELISA)...
December 1, 2016: Scandinavian Journal of Gastroenterology
https://www.readbyqxmd.com/read/27904047/protective-effect-of-deer-bone-oil-on-cartilage-destruction-in-rats-with-monosodium-iodoacetate-mia-induced-osteoarthritis
#8
Hyeon-Son Choi, Suji Im, Je Won Park, Hyung Joo Suh
The anti-osteoarthritic activity of the methanol fraction of deer bone oil extract (DBO-M) was evaluated in interleukin (IL)-1β-inflamed primary rabbit chondrocytes and in rats with monosodium iodoacetate (MIA)-induced osteoarthritis. The active compound in DBO-M was analyzed using a direct infusion liquid chromatography quadrupole (LCQ) ion-trap electrospray ionization (ESI)-mass spectrometer (MS). DBO-M significantly suppressed the IL-1β-induced sulfated-glycosaminoglycan (s-GAG) release from chondrocyte, and lowered mRNA levels of the collagen-degrading enzymes matrix metalloproteinase (MMP)-1 and MMP-3 in a dose-dependent manner...
2016: Biological & Pharmaceutical Bulletin
https://www.readbyqxmd.com/read/27888692/mitochondrial-gsh-replenishment-as-a-potential-therapeutic-approach-for-niemann-pick-type-c-disease
#9
Sandra Torres, Nuria Matías, Anna Baulies, Susana Nuñez, Cristina Alarcon-Vila, Laura Martinez, Natalia Nuño, Anna Fernandez, Joan Caballeria, Thierry Levade, Alba Gonzalez-Franquesa, Pablo Garcia-Rovés, Elisa Balboa, Silvana Zanlungo, Gemma Fabrías, Josefina Casas, Carlos Enrich, Carmen Garcia-Ruiz, José C Fernández-Checa
Niemann Pick type C (NPC) disease is a progressive lysosomal storage disorder caused by mutations in genes encoding NPC1/NPC2 proteins, characterized by neurological defects, hepatosplenomegaly and premature death. While the primary biochemical feature of NPC disease is the intracellular accumulation of cholesterol and gangliosides, predominantly in endolysosomes, mitochondrial cholesterol accumulation has also been reported. As accumulation of cholesterol in mitochondria is known to impair the transport of GSH into mitochondria, resulting in mitochondrial GSH (mGSH) depletion, we investigated the impact of mGSH recovery in NPC disease...
November 20, 2016: Redox Biology
https://www.readbyqxmd.com/read/27885808/vp1-the-major-capsid-protein-of-the-mouse-polyomavirus-binds-microtubules-promotes-their-acetylation-and-blocks-the-host-cell-cycle
#10
Lenka Horníková, Martin Fraiberk, Petr Man, Václav Janovec, Jitka Forstová
VP1, the major structural protein of the mouse polyomavirus (MPyV), is the major architectural component of the viral capsid. Its pentamers are able to self-assemble into capsid-like particles and to non-specifically bind DNA. Surface loops of the protein interact with sialic acid of ganglioside receptors. Although the replication cycle of the virus, including virion morphogenesis, proceeds in the cell nucleus, a substantial fraction of the protein is detected in the cytoplasm of late-phase MPyV-infected cells...
November 25, 2016: FEBS Journal
https://www.readbyqxmd.com/read/27873002/multimodal-detection-of-gm2-and-gm3-lipid-species-in-the-brain-of-mucopolysaccharidosis-type-ii-mouse-by-serial-imaging-mass-spectrometry-and-immunohistochemistry
#11
Martin Dufresne, Daniel Guneysu, Nathan Heath Patterson, Mieczyslaw Martin Marcinkiewicz, Anthony Regina, Michel Demeule, Pierre Chaurand
Mucopolysaccharidosis type II (Hunter's disease) mouse model (IdS-KO) was investigated by both imaging mass spectrometry (IMS) and immunohistochemistry (IHC) performed on the same tissue sections. For this purpose, IdS-KO mice brain sections were coated with sublimated 1,5-diaminonaphtalene and analyzed by high spatial resolution IMS (5 μm) and anti-GM3 IHC on the same tissue sections to characterize the ganglioside monosialated ganglioside (GM) deposits found in Hunter's disease. IMS analysis have found that two species of GM3 and GM2 that are only different due to the length of their fatty acid residue (stearic or arachidic residue) were overexpressed in the IdS-KO mice compared to a control mouse...
November 21, 2016: Analytical and Bioanalytical Chemistry
https://www.readbyqxmd.com/read/27870891/oxldl-and-eldl-induced-membrane-microdomains-in-human-macrophages
#12
Stefan Wallner, Margot Grandl, Gerhard Liebisch, Markus Peer, Evelyn Orsó, Alexander Sigrüner, Andrzej Sobota, Gerd Schmitz
BACKGROUND: Extravasation of macrophages and formation of lipid-laden foam cells are key events in the development and progression of atherosclerosis. The degradation of atherogenic lipoproteins subsequently leads to alterations in cellular lipid metabolism that influence inflammatory signaling. Especially sphingolipids and ceramides are known to be involved in these processes. We therefore analyzed monocyte derived macrophages during differentiation and after loading with enzymatically (eLDL) and oxidatively (oxLDL) modified low-density lipoproteins (LDL)...
2016: PloS One
https://www.readbyqxmd.com/read/27866733/review-of-the-literature-articles-published-in-the-last-five-years-that-have-changed-my-daily-practice
#13
REVIEW
E Delmont
Over the last five years, the management of peripheral neuropathies has become structured by the publication of recognized diagnostic criteria for inflammatory neuropathies and the elaboration of a function score, the R-ODS, used to evaluate the progression of these neuropathies. The concept of nodo-paranodopathy has enriched the concept of peripheral neuropathies, over-riding the classical mechanisms of axonal and demyelinating mechanisms. The structures of the nodes of Ranvier, gangliosides, contractin and neurofascin are preferential targets for auto-antibodies responsible for dysimmune neuropathies...
December 2016: Revue Neurologique
https://www.readbyqxmd.com/read/27857121/guillain-barr%C3%A3-syndrome-a-century-of-progress
#14
REVIEW
John A Goodfellow, Hugh J Willison
In 1916, Guillain, Barré and Strohl reported on two cases of acute flaccid paralysis with high cerebrospinal fluid protein levels and normal cell counts - novel findings that identified the disease we now know as Guillain-Barré syndrome (GBS). 100 years on, we have made great progress with the clinical and pathological characterization of GBS. Early clinicopathological and animal studies indicated that GBS was an immune-mediated demyelinating disorder, and that severe GBS could result in secondary axonal injury; the current treatments of plasma exchange and intravenous immunoglobulin, which were developed in the 1980s, are based on this premise...
November 18, 2016: Nature Reviews. Neurology
https://www.readbyqxmd.com/read/27834894/temporal-changes-of-human-breast-milk-lipids-of-chinese-mothers
#15
Francesca Giuffrida, Cristina Cruz-Hernandez, Emmanuelle Bertschy, Patric Fontannaz, Isabelle Masserey Elmelegy, Isabelle Tavazzi, Cynthia Marmet, Belén Sanchez-Bridge, Sagar K Thakkar, Carlos Antonio De Castro, Gerard Vynes-Pares, Yumei Zhang, Peiyu Wang
Fatty acids (FA), phospholipids (PL), and gangliosides (GD) play a central role in infant growth, immune and inflammatory responses. The aim of this study was to determine FA, PL, and GD compositional changes in human milk (HM) during lactation in a large group of Chinese lactating mothers (540 volunteers) residing in Beijing, Guangzhou, and Suzhou. HM samples were collected after full expression from one breast and while the baby was fed on the other breast. FA were assessed by direct methylation followed by gas chromatography (GC) analysis...
November 10, 2016: Nutrients
https://www.readbyqxmd.com/read/27832917/immune-response-characterization-and-vaccine-potential-of-a-recombinant-chimera-comprising-b-cell-epitope-of-aeromonas-hydrophila-outer-membrane-protein-c-and-ltb
#16
Mahima Sharma, Aparna Dixit
Aeromonas hydrophila is one of the most virulent fish pathogens, causing colossal economic losses to the aquaculture industry annually. The absence of a safe and effective vaccine makes it very difficult to control this infection. Outer membrane proteins have been widely illustrated to confer protective immunity against a broad spectrum of gram negative bacteria. In the current study, we have analyzed the ability of B-cell epitopes of A. hydrophila's outer membrane protein C (OmpC) to confer protection against bacterial virulence...
December 7, 2016: Vaccine
https://www.readbyqxmd.com/read/27832720/application-study-of-1-2-%C3%AE-l-fucosynthase-introduction-of-fuc%C3%AE-1-2gal-disaccharide-structures-on-n-glycan-ganglioside-and-xyloglucan-oligosaccharide
#17
Yuta Sugiyama, Toshihiko Katoh, Yuji Honda, Aina Gotoh, Hisashi Ashida, Shin Kurihara, Kenji Yamamoto, Takane Katayama
We have recently generated a highly efficient 1,2-α-l-fucosynthase (BbAfcA N423H mutant) by protein engineering of 1,2-α-l-fucosidase from Bifidobacterium bifidum JCM 1254. This synthase could specifically introduce H-antigens (Fucα1-2Gal) into the non-reducing ends of oligosaccharides and in O-linked glycans in mucin glycoprotein. In the present study, we show an extended application of the engineered 1,2-α-l-fucosynthase by demonstrating its ability to insert Fuc residues into N- and O-glycans in fetuin glycoproteins, GM1 ganglioside, and a plant-derived xyloglucan nonasaccharide...
November 11, 2016: Bioscience, Biotechnology, and Biochemistry
https://www.readbyqxmd.com/read/27832592/receptor-mediated-sorting-of-typhoid-toxin-during-its-export-from-salmonella-typhi-infected-cells
#18
Shu-Jung Chang, Jeongmin Song, Jorge E Galán
Typhoid toxin is an essential virulence factor of Salmonella Typhi, the cause of typhoid fever. Typhoid toxin is secreted into the lumen of Salmonella-containing vacuole (SCV), after which it is packaged into vesicle carrier intermediates and released extracellularly through incompletely understood mechanisms. Following export, the toxin targets cells by interacting with human-specific Neu5Ac-terminated glycan receptors. We show that typhoid toxin is sorted from the SCV into vesicle carrier intermediates via interactions of its B subunit, PltB, with specific lumenal sialylated glycan packaging receptors...
November 9, 2016: Cell Host & Microbe
https://www.readbyqxmd.com/read/27832416/slow-continuous-enzyme-replacement-via-spinal-csf-in-dogs-with-the-paediatric-onset-neurodegenerative-disease-mps-iiia
#19
Barbara King, Neil R Marshall, Sofia Hassiotis, Paul J Trim, Justin Tucker, Kathryn Hattersley, Marten F Snel, Robert D Jolly, John J Hopwood, Kim M Hemsley
Intra-cerebrospinal fluid (CSF) injection of recombinant human lysosomal enzyme is a potential treatment strategy for several neurodegenerative lysosomal storage disorders including Sanfilippo syndrome (Mucopolysaccharidosis type IIIA; MPS IIIA). Here we have utilised the MPS IIIA Huntaway dog model to compare the effectiveness of the repeated intermittent bolus injection strategy being used in the trials with an alternate approach; slow, continual infusion of replacement enzyme (recombinant human sulphamidase; rhSGSH) into the spinal CSF using a SynchroMed II® pump attached to a spinal infusion cannula...
November 10, 2016: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/27823807/electrophysiological-assessment-of-guillain-barr%C3%A3-syndrome-with-both-gal-c-and-ganglioside-antibodies-tendency-for-demyelinating-type
#20
Makoto Samukawa, Motoi Kuwahara, Miyuki Morikawa, Rino Ueno, Yukihiro Hamada, Kazuo Takada, Makito Hirano, Yoshiyuki Mitsui, Masahiro Sonoo, Susumu Kusunoki
Whether patients who have GBS with antibodies to galactocerebroside (Gal-C) and gangliosides (Gal-C-GS-GBS) more often have demyelinating or axonal neuropathy remains controversial. We assessed the electrophysiological data from 16 patients with Gal-C-GS-GBS based on the two established criteria to clarify this issue. In this largest cohort of Gal-C-GS-GBS, eight patients had demyelinating neuropathy and none exhibited axonal neuropathy on either criterion. These data indicated that antibodies to Gal-C, a myelin antigen, might predominantly be associated with demyelinating neuropathy, even in the presence of concomitant antibodies to gangliosides...
October 24, 2016: Journal of Neuroimmunology
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