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https://www.readbyqxmd.com/read/29343457/sphingolipid-signaling-in-renal-fibrosis
#1
REVIEW
Andrea Huwiler, Josef Pfeilschifter
Over the last decade, various sphingolipid subspecies have gained increasing attention as important signaling molecules that regulate a multitude of physiological and pathophysiological processes including inflammation and tissue remodeling. These mediators include ceramide, sphingosine 1-phosphate (S1P), the cerebroside glucosylceramide, lactosylceramide, and the gangliosides GM3 and Gb3. These lipids have been shown to accumulate in various chronic kidney diseases that typically end in renal fibrosis and ultimately renal failure...
January 14, 2018: Matrix Biology: Journal of the International Society for Matrix Biology
https://www.readbyqxmd.com/read/29340649/optic-nerve-regeneration-after-crush-remodels-the-injury-site-molecular-insights-from-imaging-mass-spectrometry
#2
David T Stark, David M G Anderson, Jacky M K Kwong, Nathan Heath Patterson, Kevin L Schey, Richard M Caprioli, Joseph Caprioli
Purpose: Mammalian central nervous system axons fail to regenerate after injury. Contributing factors include limited intrinsic growth capacity and an inhibitory glial environment. Inflammation-induced optic nerve regeneration (IIR) is thought to boost retinal ganglion cell (RGC) intrinsic growth capacity through progrowth gene expression, but effects on the inhibitory glial environment of the optic nerve are unexplored. To investigate progrowth molecular changes associated with reactive gliosis during IIR, we developed an imaging mass spectrometry (IMS)-based approach that identifies discriminant molecular signals in and around optic nerve crush (ONC) sites...
January 1, 2018: Investigative Ophthalmology & Visual Science
https://www.readbyqxmd.com/read/29337417/guillain-barr%C3%A3-syndrome-in-a-local-area-in-japan-2006-2015-an-epidemiological-and-clinical-study-of-108-patients
#3
Naoko Matsui, Hiroyuki Nodera, Daisuke Kuzume, Naoki Iwasa, Yuki Unai, Waka Sakai, Yoshimichi Miyazaki, Hiroki Yamazaki, Yusuke Osaki, Takahiro Furukawa, Masahiro Yamasaki, Yuishin Izumi, Susumu Kusunoki, Kokichi Arisawa, Ryuji Kaji
BACKGROUND: Many epidemiological studies of Guillain-Barré syndrome (GBS) and Fisher syndrome (FS) have been conducted in Europe and America. In contrast, epidemiological studies are rare in Asia where the GBS subtypes differ from those in Western countries. This study was undertaken to clarify the incidence of GBS and FS in a local area in Japan as well as their seasonal trends. METHODS: Seventy-one GBS and 37 FS patients were recorded from 2006 to 2015 in an area of approximately 1...
January 16, 2018: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/29331587/il-23-il-17-immune-axis-in-guillain-barr%C3%A3-syndrome-exploring-newer-vistas-for-understanding-pathobiology-and-therapeutic-implications
#4
REVIEW
Monojit Debnath, Madhu Nagappa, Geetanjali Murari, Arun B Taly
Guillain Barré Syndrome (GBS) is a severe disorder of the peripheral nervous system with an inadequately known etiopathology. It is a post infectious immune mediated disorder, characterized by autoantibody production, complement activation as well as T reactivity against gangliosides. However, the precise etiopathogenesis remains poorly understood in a majority of the patients. Th17 cells, a recently identified lineage of Th cells have emerged as a predominant inducer of autoimmunity and inflammation in various immunological disorders...
January 10, 2018: Cytokine
https://www.readbyqxmd.com/read/29329103/analysis-of-anti-ganglioside-antibodies-by-a-line-immunoassay-in-patients-with-chronic-inflammatory-demyelinating-polyneuropathies-cidp
#5
Juliane Klehmet, Stefanie Märschenz, Klemens Ruprecht, Benjamin Wunderlich, Thomas Büttner, Rico Hiemann, Dirk Roggenbuck, Andreas Meisel
BACKGROUND: Unlike for acute immune-mediated neuropathies (IN), anti-ganglioside autoantibody (aGAAb) testing has been recommended for only a minority of chronic IN yet. Thus, we used a multiplex semi-quantitative line immunoassay (LIA) to search for aGAAb in chronic-inflammatory demyelinating polyneuropathy (CIDP) and its clinical variants. METHODS: Anti-GAAb to 11 gangliosides and sulfatide (SF) were investigated by LIA in 61 patients with IN (27 typical CIDP, 12 distal-acquired demyelinating polyneuropathy, 6 multifocal-acquired demyelinating sensory/motor polyneuropathy, 10 sensory CIDP, 1 focal CIDP and 5 multifocal-motoric neuropathy), 40 with other neuromuscular disorders (OND) (15 non-immune polyneuropathies, 25 myasthenia gravis), 29 with multiple sclerosis (MS) and 54 healthy controls (HC)...
January 12, 2018: Clinical Chemistry and Laboratory Medicine: CCLM
https://www.readbyqxmd.com/read/29319803/down-regulation-of-glutamate-release-from-hippocampal-neurons-by-sialidase
#6
Minami Akira, Ami Ishii, Sumika Shimba, Takahiro Kano, Eri Fujioka, Saki Sai, Nagisa Oshio, Sayaka Ishibashi, Tadanobu Takahashi, Yuuki Kurebayashi, Hiroaki Kanazawa, Nobuhiro Yuki, Tadamune Otsubo, Kiyoshi Ikeda, Takashi Suzuki
Sialidase, which removes sialic acid residues in sialylglycoconjugates, is essential for hippocampal memory and synaptic plasticity. Enzyme activity of sialidase is rapidly increased in response to neural excitation. Because sialic acid bound to gangliosides such as the tetra-sialoganglioside GQ1b is crucial for calcium signaling and neurotransmitter release, neural activity-dependent removal of sialic acid may affect hippocampal neurotransmission. In the present study, we found that 2-deoxy-2,3-didehydro-D-N-acetylneuraminic acid (DANA), a sialidase inhibitor, increased expression of ganglioside GQ1b/GT1a in hippocampal acute slices...
January 8, 2018: Journal of Biochemistry
https://www.readbyqxmd.com/read/29317695/alterations-in-the-properties-of-the-cell-membrane-due-to-glycosphingolipid-accumulation-in-a-model-of-gaucher-disease
#7
Gyula Batta, Lilla Soltész, Tamás Kovács, Tamás Bozó, Zoltán Mészár, Miklós Kellermayer, János Szöllősi, Peter Nagy
Gaucher disease is a lysosomal storage disease characterized by the malfunction of glucocerebrosidase resulting in the accumulation of glucosylceramide and other sphingolipids in certain cells. Although the disease symptoms are usually attributed to the storage of undigested substrate in lysosomes, here we show that glycosphingolipids accumulating in the plasma membrane cause profound changes in the properties of the membrane. The fluidity of the sphingolipid-enriched membrane decreased accompanied by the enlargement of raft-like ordered membrane domains...
January 9, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29315669/guillain-barr%C3%A3-syndrome-subtype-diagnosis-a-prospective-multicentric-european-study
#8
Peter Y K Van den Bergh, Françoise Piéret, John L Woodard, Shahram Attarian, Aude-Marie Grapperon, Guillaume Nicolas, Marion Brisset, Julien Cassereau, Yusuf A Rajabally, Vinciane Van Parijs, Donatienne Verougstraete, Philippe Jacquerye, Jean-Marc Raymackers, Céline Redant, Claure Michel, Emilien Delmont
INTRODUCTION: There is uncertainty as to whether the Guillain-Barré syndrome (GBS) subtypes, acute inflammatory demyelinating polyradiculoneuropathy (AIDP) and acute motor axonal neuropathy (AMAN), can be diagnosed electrophysiologically. METHODS: We prospectively included 58 GBS patients. Electrophysiology was performed at means of 5 and 33 days after disease onset. Two traditional and one recent criteria sets were used to classify studies as demyelinating or axonal...
January 5, 2018: Muscle & Nerve
https://www.readbyqxmd.com/read/29311330/gba1-deficiency-negatively-affects-physiological-%C3%AE-synuclein-tetramers-and-related-multimers
#9
Sangjune Kim, Seung Pil Yun, Saebom Lee, George Essien Umanah, Veera Venkata Ratnam Bandaru, Xiling Yin, Peter Rhee, Senthilkumar S Karuppagounder, Seung-Hwan Kwon, Hojae Lee, Xiaobo Mao, Donghoon Kim, Akhilesh Pandey, Gabsang Lee, Valina L Dawson, Ted M Dawson, Han Seok Ko
Accumulating evidence suggests that α-synuclein (α-syn) occurs physiologically as a helically folded tetramer that resists aggregation. However, the mechanisms underlying the regulation of formation of α-syn tetramers are still mostly unknown. Cellular membrane lipids are thought to play an important role in the regulation of α-syn tetramer formation. Since glucocerebrosidase 1 (GBA1) deficiency contributes to the aggregation of α-syn and leads to changes in neuronal glycosphingolipids (GSLs) including gangliosides, we hypothesized that GBA1 deficiency may affect the formation of α-syn tetramers...
January 8, 2018: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/29307477/enhancing-toxin-based-vaccines-against-botulism
#10
Amanda Przedpelski, William H Tepp, Madison Zuverink, Eric A Johnson, Sabine Pellet, Joseph T Barbieri
Botulinum neurotoxins (BoNT) are the most toxic proteins for humans. BoNTs are single chain proteins with an N-terminal light chain (LC) and a C-terminal heavy chain (HC). HC comprises a translocation domain (HCN) and a receptor binding domain (HCC). Currently, there are no approved vaccines against botulism. This study tests a recombinant, full-length BoNT/A1 versus LCHCN/A1 and HCC/A1 as vaccine candidates against botulism. Recombinant, full-length BoNT/A1 was detoxified by engineering 3-amino acid mutations (E224A/R363A/Y366F) (M-BoNT/A1) into the LC to eliminate catalytic activity, which reduced toxicity in a mouse model of botulism by >106-fold relative to native BoNT/A1...
January 4, 2018: Vaccine
https://www.readbyqxmd.com/read/29306858/guillain-barr%C3%A3-syndrome-with-exaggerated-pleocytosis-and-anti-gm1-ganglioside-antibodies
#11
Gabriel T Doctor, Sian K Alexander, Aleksandar Radunovic
An 81-year-old man presented with fever, confusion and rapidly-progressive flaccid tetraparesis. Clinical presentation and neurophysiology were consistent with a severe axonal polyneuropathy. Anti-GM1 and Campylobacter serology were both positive, consistent with postinfectious axonal-variant Guillain-Barré syndrome (GBS). GBS is characterised by albuminocytological dissociation, where an elevated protein and acellular cerebrospinal fluid are typical. However, in this case, CSF analysis revealed an exaggerated pleocytosis (72 white blood cells (WBC)/mm3)...
January 6, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29306438/revealing-the-raft-domain-organization-in-the-plasma-membrane-by-single-molecule-imaging-of-fluorescent-ganglioside-analogs
#12
Kenichi G N Suzuki, Hiromune Ando, Naoko Komura, Miku Konishi, Akihiro Imamura, Hideharu Ishida, Makoto Kiso, Takahiro K Fujiwara, Akihiro Kusumi
Gangliosides have been implicated in a variety of physiological processes, particularly in the formation and function of raft domains in the plasma membrane. However, the scarcity of suitable fluorescent ganglioside analogs had long prevented us from determining exactly how gangliosides perform their functions in the live-cell plasma membrane. With the development of new fluorescent ganglioside analogs, as described by Komura et al. (2017), this barrier has been broken. We can now address the dynamic behaviors of gangliosides in the live-cell plasma membrane, using fluorescence microscopy, particularly by single-fluorescent molecule imaging and tracking...
2018: Methods in Enzymology
https://www.readbyqxmd.com/read/29304106/bovine-dairy-complex-lipids-improve-in-vitro-measures-of-small-intestinal-epithelial-barrier-integrity
#13
Rachel C Anderson, Alastair K H MacGibbon, Neill Haggarty, Kelly M Armstrong, Nicole C Roy
Appropriate intestinal barrier maturation is essential for absorbing nutrients and preventing pathogens and toxins from entering the body. Compared to breast-fed infants, formula-fed infants are more susceptible to barrier dysfunction-associated illnesses. In infant formula dairy lipids are usually replaced with plant lipids. We hypothesised that dairy complex lipids improve in vitro intestinal epithelial barrier integrity. We tested milkfat high in conjugated linoleic acid, beta serum (SureStart™Lipid100), beta serum concentrate (BSC) and a ganglioside-rich fraction (G600)...
2018: PloS One
https://www.readbyqxmd.com/read/29301656/cd1a-and-cd1e-gene-polymorphisms-are-not-associated-with-susceptibility-to-guillain-barr%C3%A3-syndrome-in-the-bangladeshi-population
#14
Mohammad I Rahman, Iffat Jahan, Mir M Khalid, Israt Jahan, Rijwan U Ahammad, Shamsun Nahar, Zhahirul Islam
The post-infectious autoimmune polyradiculoneuropathy Guillain-Barré syndrome (GBS) is triggered by molecular mimicry between microbial glycolipid antigens and human peripheral nerve gangliosides. Single nucleotide polymorphisms in exon 2 of CD1A (*01/*02) and CD1E (*01/*02) were assessed using PCR-RFLP; no significant differences in genotype or allele frequency were observed between 200 patients with GBS and 200 healthy controls. CD1 gene polymorphisms cannot be recognized as a susceptibility or disease-causative factor for GBS in the Bangladeshi population...
January 15, 2018: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/29298689/anti-gd2-4-1bb-chimeric-antigen-receptor-t-cell-therapy-for-the-treatment-of-chinese-melanoma-patients
#15
Jiayi Yu, Xiaowen Wu, Junya Yan, Huan Yu, Longwen Xu, Zhihong Chi, Xinan Sheng, Lu Si, Chuanliang Cui, Jie Dai, Meng Ma, Tianxiao Xu, Yan Kong, Jun Guo
BACKGROUND: Chimeric antigen receptor (CAR)-engineered T cells have demonstrated promising clinical efficacy in patients with B cell lymphoma. However, the application of CAR-T cell therapy in the treatment of other solid tumors has been limited. We incorporated 4-1BB into the anti-GD2 CAR-T cells to test their cytotoxicity in melanoma in vitro and in vivo. Moreover, we reported the expression of ganglioside GD2 in non-Caucasian melanoma populations for the first time, thus providing a basis for future clinical research...
January 3, 2018: Journal of Hematology & Oncology
https://www.readbyqxmd.com/read/29288365/methylphenidate-disrupts-cytoskeletal-homeostasis-and-reduces-membrane-associated-lipid-content-in-juvenile-rat-hippocampus
#16
Felipe Schmitz, Paula Pierozan, Helena Biasibetti-Brendler, Fernanda Silva Ferreira, Fernanda Dos Santos Petry, Vera Maria Treis Trindade, Regina Pessoa-Pureur, Angela T S Wyse
Although methylphenidate (MPH) is ubiquitously prescribed to children and adolescents, the consequences of chronic utilization of this psychostimulant are poorly understood. In this study, we investigated the effects of MPH on cytoskeletal homeostasis and lipid content in rat hippocampus. Wistar rats received intraperitoneal injections of MPH (2.0 mg/kg) or saline solution (controls), once a day, from the 15th to the 44th day of age. Results showed that MPH provoked hypophosphorylation of glial fibrillary acidic protein (GFAP) and reduced its immunocontent...
December 29, 2017: Metabolic Brain Disease
https://www.readbyqxmd.com/read/29287682/development-of-an-fhbp-ctb-holotoxin-like-chimera-and-the-elicitation-of-bactericidal-antibodies-against-serogroup-b-neisseria-meningitidis
#17
Gregory A Price, Margaret C Bash
The Neisseria meningitidis factor H binding protein (FHbp) is an important virulence factor and vaccine antigen contained in both USA licensed serogroup B meningococcal vaccines. Recent studies in human factor H (hFH) transgenic mice suggest that hFH-FHbp interactions lower FHbp-elicited immunogenicity. To provide tools with which to characterize and potentially improve FHbp immunogenicity, we developed an FHbp-cholera holotoxin-like chimera vaccine expression system in Escherichia coli that utilizes cholera toxin B (CTB) as both a scaffold and adjuvant for FHbp...
December 26, 2017: Vaccine
https://www.readbyqxmd.com/read/29282205/glycosphingolipid-metabolic-reprogramming-drives-neural-differentiation
#18
Domenico Russo, Floriana Della Ragione, Riccardo Rizzo, Eiji Sugiyama, Francesco Scalabrì, Kei Hori, Serena Capasso, Lucia Sticco, Salvatore Fioriniello, Roberto De Gregorio, Ilaria Granata, Mario R Guarracino, Vittorio Maglione, Ludger Johannes, Gian Carlo Bellenchi, Mikio Hoshino, Mitsutoshi Setou, Maurizio D'Esposito, Alberto Luini, Giovanni D'Angelo
Neural development is accomplished by differentiation events leading to metabolic reprogramming. Glycosphingolipid metabolism is reprogrammed during neural development with a switch from globo- to ganglio-series glycosphingolipid production. Failure to execute this glycosphingolipid switch leads to neurodevelopmental disorders in humans, indicating that glycosphingolipids are key players in this process. Nevertheless, both the molecular mechanisms that control the glycosphingolipid switch and its function in neurodevelopment are poorly understood...
December 27, 2017: EMBO Journal
https://www.readbyqxmd.com/read/29273615/longitudinally-extensive-transverse-myelitis-after-campylobacter-jejuni-enteritis
#19
Yudy Llamas, Karl Hazel, Patrick Nicholson, Lisa Costelloe
Campylobacter jejuni infection is well-known to precipitate Guillain-Barré syndrome through an immune-mediated attack on the peripheral nervous system. Molecular mimicry between C. jejuni lipo-oligosaccharides on the surface of infectious agents and human gangliosides in the peripheral nerves induces cross-reactive immune responses. Although gangliosides also occur in the central nervous system (CNS), autoimmune CNS disorders rarely follow Cjejuni infections. However, longitudinally extensive transverse myelitis commonly has a parainfectious cause, triggered by a wide range of micro-organisms including viruses and bacteria...
December 22, 2017: Practical Neurology
https://www.readbyqxmd.com/read/29248893/autoimmune-nodo-paranodopathies-of-peripheral-nerve-the-concept-is-gaining-ground
#20
REVIEW
Antonino Uncini, Jean-Michel Vallat
Peripheral neuropathies are classified as primarily demyelinating or axonal. Microstructural alterations of the nodal region are the key to understand the pathophysiology of neuropathies with antibodies to gangliosides and the new category of nodo-paranodopathy has been proposed to better characterise these disorders and overcome some inadequacies of the dichotomous classification. Recently, the research in autoimmune neuropathies has been boosted by reports of patients carrying immunoglobulin G4 antibodies against paranodal axo-glial proteins with distinct phenotypes and showing loss of transverse bands, terminal myelin loop detachment, nodal widening and axonal loss...
December 16, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
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