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https://www.readbyqxmd.com/read/29352662/distinct-progression-patterns-of-brain-disease-in-infantile-and-juvenile-gangliosidoses-volumetric-quantitative-mri-study
#1
Igor Nestrasil, Alia Ahmed, Josephine M Utz, Kyle Rudser, Chester B Whitley, Jeanine R Jarnes-Utz
BACKGROUND: GM1-gangliosidosis and GM2-gangliosidosis (Tay-Sachs disease and Sandhoff disease) are unrelenting heritable neurodegenerative conditions of lysosomal ganglioside accumulation. Although progressive brain atrophy is characteristic, longitudinal quantification of specific brain structures has not been systematically studied. OBJECTIVES: The goal of this longitudinal study has been to quantify and track brain MRI volume changes, including specific structure volume changes, at different times in disease progression of childhood gangliosidoses, and to explore quantitative brain MRI volumetry (qMRI) as a non-invasive marker of disease progression for future treatment trials...
December 20, 2017: Molecular Genetics and Metabolism
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
#2
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/29317695/alterations-in-the-properties-of-the-cell-membrane-due-to-glycosphingolipid-accumulation-in-a-model-of-gaucher-disease
#3
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/29311511/a-peptide-glycolipid-interaction-probed-by-retroinverso-peptide-analogues
#4
Kaori Sakurai
Cell surface glycolipids are implicated in the formation of lipid rafts and membrane microdomains, where they interact with protein receptors to mediate a variety of cellular processes such as cell-cell recognition, cell adhesion, and membrane signaling. Studies of glycolipid function at the local membrane structures have not been straightforward to date, because the locally clustered structures are labile and their protein binding affinities tend to be weak. While specific glycolipid-binding proteins have been employed as molecular probes for detecting lipid rafts, small peptides may be more suitable for probing glycolipids at the cell surface due to their small size as well as their ease of synthetic preparation and functionalization...
2018: Chemical & Pharmaceutical Bulletin
https://www.readbyqxmd.com/read/29306858/guillain-barr%C3%A3-syndrome-with-exaggerated-pleocytosis-and-anti-gm1-ganglioside-antibodies
#5
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
#6
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/29288365/methylphenidate-disrupts-cytoskeletal-homeostasis-and-reduces-membrane-associated-lipid-content-in-juvenile-rat-hippocampus
#7
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
#8
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/29221755/unilateral-brachial-plexopathy-a-rare-complication-of-mycoplasma-pneumoniae-infection
#9
Barbara Robnik, Darja Keše, Tereza Rojko, Alenka Horvat-Ledinek, Aleš Pražnikar, Bojana Beović
Few reports in the literature describe isolated peripheral neuropathies in relation to Mycoplasma pneumoniae infection without concurrent damage to the central nervous system. To our knowledge only a single case of mononeuritis multiplex with brachial plexus neuropathy coincident with M. pneumoniae has been documented until now. Here we present the first clinical case of lobar M. pneumoniae pneumonia in a 19-year-old female patient, where coincident neurological complications manifested as unilateral brachial plexus neuropathy, affecting axillar and suprascapular nerves...
December 5, 2017: Journal of Infection and Chemotherapy: Official Journal of the Japan Society of Chemotherapy
https://www.readbyqxmd.com/read/29170521/age-dependent-and-regional-heterogeneity-in-the-long-chain-base-of-a-series-gangliosides-observed-in-the-rat-brain-using-maldi-imaging
#10
Sarah Caughlin, Shikhar Maheshwari, Nina Weishaupt, Ken K-C Yeung, David Floyd Cechetto, Shawn Narain Whitehead
Alterations in the long chain base of the sphingosine moiety of gangliosides have been shown to play a role in neurodevelopment and neurodegeneration. Indeed, the accumulation of d20:1 sphingosine has been referred to as a metabolic marker of aging in the brain, however, this remains to be shown in simple gangliosides GM2 and GM3. In this study, Matrix-assisted laser desorption/ionization Imaging Mass Spectrometry (MALDI IMS) was used to examine the neuroanatomical distribution of A-series gangliosides with either 18 or 20 carbon sphingosine chains (d18:1 or d20:1) in Fisher 344 rats across the lifespan...
November 23, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29163491/suppression-of-natural-killer-cell-activity-by-regulatory-nkt10-cells-aggravates-alcoholic-hepatosteatosis
#11
Kele Cui, Guoxiu Yan, Xiaodong Zheng, Li Bai, Haiming Wei, Rui Sun, Zhigang Tian
We and others have found that the functions of hepatic natural killer (NK) cells are inhibited but invariant NKT (iNKT) cells become activated after alcohol drinking, leaving a possibility that there exists interplay between NK cells and iNKT cells during alcoholic liver disease. Here, in a chronic plus single-binge ethanol consumption mouse model, we observed that NK cells and interferon-γ (IFN-γ) protected against ethanol-induced liver steatosis, as both wild-type (WT) mice treated with anti-asialo GM1 antibody and IFN-γ-deficient GKO mice developed more severe alcoholic fatty livers...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/29160035/mosaic-uniparental-disomy-results-in-gm1-gangliosidosis-with-normal-enzyme-assay
#12
Kenneth A Myers, Mark F Bennett, Chung W Chow, Susan M Carden, Simone A Mandelstam, Melanie Bahlo, Ingrid E Scheffer
Inherited metabolic disorders are traditionally diagnosed using broad and expensive panels of screening tests, often including invasive skin and muscle biopsy. Proponents of next-generation genetic sequencing have argued that replacing these screening panels with whole exome sequencing (WES) would save money. Here, we present a complex patient in whom WES allowed diagnosis of GM1 gangliosidosis, caused by homozygous GLB1 mutations, resulting in β-galactosidase deficiency. A 10-year-old girl had progressive neurologic deterioration, macular cherry-red spot, and cornea verticillata...
November 21, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/29148800/size-and-shape-of-amyloid-fibrils-induced-by-ganglioside-nanoclusters-role-of-sialyl-oligosaccharide-in-fibril-formation
#13
Teruhiko Matsubara, Masaya Nishihara, Hanaki Yasumori, Mako Nakai, Katsuhiko Yanagisawa, Toshinori Sato
Ganglioside-enriched microdomains in the presynaptic neuronal membrane play a key role in the initiation of amyloid ß-protein (Aß) assembly related to Alzheimer's disease. We previously isolated lipids from a detergent-resistant membrane microdomain fraction of synaptosomes prepared from aged mouse brain and found that spherical Aß assemblies were formed on Aß-sensitive ganglioside nanoclusters (ASIGN) of reconstituted lipid bilayers in the synaptosomal fraction. In the present study, we investigated the role of oligosaccharides in Aß fibril formation induced by ganglioside-containing mixed lipid membranes that mimic the features of ASIGN...
November 17, 2017: Langmuir: the ACS Journal of Surfaces and Colloids
https://www.readbyqxmd.com/read/29136715/-analysis-of-the-clinical-and-electrophysiological-characteristics-of-multifocal-motor-neuropathy
#14
H F Wang, F Yang, F Cui, Z H Chen, L Ling, X S Huang
Ten patients diagnosed with multifocal motor neuropathy (MMN) were recruited in the Department of Neurology at Chinese PLA General Hospital from January 1, 2009 to August 31, 2015. The clinical and electrophysiological features were analyzed retrospectively. All patients complained of progressive asymmetric limb weakness, which was more severe in distal than in proximal. Five presented muscle atrophy. None had sensory disturbances. All suffered diminished or disappeared tendon reflex, whereas Babinski signs were negative...
November 1, 2017: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
https://www.readbyqxmd.com/read/29126773/clomipramine-counteracts-lipid-raft-disturbance-due-to-short-term-muscle-disuse
#15
Irina G Bryndina, Maria N Shalagina, Alexey V Sekunov, Andrei L Zefirov, Alexey M Petrov
Disuse-induced skeletal muscle dysfunction is a serious consequence of long-term spaceflight, numerous diseases and conditions for which treatment possibilities are still strictly limited. We have previously shown that acute hindlimb suspension (HS)-mediated disuse disrupts membrane lipid rafts in the unloaded muscle. Here, we investigated whether pretreatment of rats with the inhibitor of acid sphingomyelinase, clomipramine (1.25mg/g/day, intramuscularly, for 5days before HS), is able to hinder the loss in lipid raft integrity in response to 12h of HS...
November 7, 2017: Neuroscience Letters
https://www.readbyqxmd.com/read/29119347/n-glycan-structures-and-downstream-mannose-phosphorylation-of-plant-recombinant-human-alpha-l-iduronidase-toward-development-of-enzyme-replacement-therapy-for-mucopolysaccharidosis-i
#16
Owen M Pierce, Grant R McNair, Xu He, Hiroyuki Kajiura, Kazuhito Fujiyama, Allison R Kermode
Arabidopsis N-glycan processing mutants provide the basis for tailoring recombinant enzymes for use as replacement therapeutics to treat lysosomal storage diseases, including N-glycan mannose phosphorylation to ensure lysosomal trafficking and efficacy. Functional recombinant human alpha-L-iduronidase (IDUA; EC 3.2.1.76) enzymes were generated in seeds of the Arabidopsis thaliana complex-glycan-deficient (cgl) C5 background, which is deficient in the activity of N-acetylglucosaminyl transferase I, and in seeds of the Arabidopsis gm1 mutant, which lacks Golgi α-mannosidase I (GM1) activity...
November 8, 2017: Plant Molecular Biology
https://www.readbyqxmd.com/read/29112834/natural-killer-cells-play-an-essential-role-in-resolution-of-antigen-induced-inflammation-in-mice
#17
Osk U U Anuforo, Stefania P Bjarnarson, Hulda S Jonasdottir, Martin Giera, Ingibjorg Hardardottir, Jona Freysdottir
This study examined whether NK cells are important for resolution of antigen-induced inflammation. C57BL/6 mice were immunized twice with methylated BSA (mBSA) and inflammation induced by intraperitoneal injection of mBSA. Mice were injected intravenously with anti-asialo GM1 (αASGM1) or a control antibody 24h prior to peritonitis induction and peritoneal exudate collected at different time points. Expression of surface molecules and apoptosis on peritoneal cells was determined by flow cytometry and concentration of chemokines, cytokines, soluble cytokine receptors and lipid mediators by ELISA and LC-MS/MS...
November 4, 2017: Molecular Immunology
https://www.readbyqxmd.com/read/29111890/polyethylene-glycol-b-poly-lactic-acid-polymersomes-as-vehicles-for-enzyme-replacement-therapy
#18
Jessica M Kelly, Amanda L Gross, Douglas R Martin, Mark E Byrne
AIM: Polymersomes are created to deliver an enzyme-based therapy to the brain in lysosomal storage disease patients. MATERIALS & METHODS: Polymersomes are formed via the injection method using poly(ethylene glycol)-b-poly(lactic acid) (PEGPLA) and bound to apolipoprotein E, to create a brain-targeted delivery vehicle. RESULTS: Polymersomes have a smallest average diameter of 145 ± 21 nm and encapsulate β-galactosidase at 72.0 ± 12...
November 7, 2017: Nanomedicine
https://www.readbyqxmd.com/read/29106974/direct-visualization-of-the-lateral-structure-of-giant-vesicles-composed-of-pseudo-binary-mixtures-of-sulfatide-asialo-gm1-and-gm1-with-popc
#19
Pablo M Rodi, Bruno Maggio, Luis A Bagatolli
We compared the lateral structure of giant unilamellar vesicles (GUVs) composed of three pseudo binary mixtures of different glycosphingolipid (GSL), i.e. sulfatide, asialo-GM1 or GM1, with POPC. These sphingolipids possess similar hydrophobic residues but differ in the size and charge of their polar head group. Fluorescence microscopy experiments using LAURDAN and DiIC18 show coexistence of micron sized domains in a molar fraction range that depends on the nature of the GSLs. In all cases, experiments with LAURDAN show that the membrane lateral structure resembles the coexistence of solid ordered and liquid disordered phases...
October 26, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/29106782/expression-and-assembly-of-largest-foreign-protein-in-chloroplasts-oral-delivery-of-human-fviii-made-in-lettuce-chloroplasts-robustly-suppresses-inhibitor-formation-in-hemophilia-a-mice
#20
Kwang-Chul Kwon, Alexandra Sherman, Wan-Jung Chang, Aditya Kamesh, Moanaro Biswas, Roland W Herzog, Henry Daniell
Inhibitor formation is a serious complication of factor VIII (FVIII) replacement therapy for the X-linked bleeding disorder hemophilia A and occurs in 20-30% of patients. No prophylactic tolerance protocol currently exists. Although we reported oral tolerance induction using FVIII domains expressed in tobacco chloroplasts, significant challenges in clinical advancement include expression of the full-length CTB-FVIII sequence to cover the entire patient population, regardless of individual CD4(+) T cell epitope responses...
November 6, 2017: Plant Biotechnology Journal
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