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https://www.readbyqxmd.com/read/28544772/ganglioside-gm3-content-in-skeletal-muscles-is-increased-in-type-2-but-decreased-in-type-1-diabetes-rat-models-implications-of-glycosphingolipid-metabolism-in-pathophysiology-of-diabetes
#1
Josko Bozic, Anita Markotic, Vedrana Cikes-Culic, Anela Novak, Josip Andelo Borovac, Hrvoje Vucemilovic, Gorana Trgo, Tina Ticinovic Kurir
BACKGROUND: Ganglioside GM3 is an abundant constituent of the plasma membrane where its accumulation attenuates insulin receptor signaling. Considering the role of skeletal muscles for insulin-stimulated glucose uptake, the aim of this study was to determine the expression of GM3 and its precursors in skeletal muscle of diabetes mellitus type 1 (DM1) and type 2 (DM2) rat models. METHODS: Diabetes was induced by streptozotocin (55 mg/kg for DM1 and 35 mg/kg for DM2) injection to male Sprague-Dawley rats which were fed with normal pellet diet (DM1 type) or high-fat diet (DM2 type)...
May 22, 2017: Journal of Diabetes
https://www.readbyqxmd.com/read/28537895/interaction-of-glycosphingolipids-gd3-and-gd2-with-growth-factor-receptors-maintains-breast-cancer-stem-cell-phenotype
#2
Yuh-Jin Liang, Chen-Yu Wang, I-An Wang, Yi-Wen Chen, Li-Tzu Li, Chuang-Yu Lin, Ming-Yi Ho, Tsung-Lung Chou, Ya-Hui Wang, Shih-Pin Chiou, Yu-Ju Lin, John Yu
Many studies have suggested that disialogangliosides, GD2 and GD3, are involved in the development of various tumor types. However, the functional relationships between ganglioside expression and cancer development or aggressiveness are not fully described. GD3 is upregulated in approximately half of all invasive ductal breast carcinoma cases, and enhanced expression of GD3 synthase (GD3S, alpha-N-acetylneuraminide alpha-2,8-sialyltransferase) in estrogen receptor-negative breast tumors, was shown to correlate with reduced overall patient survival...
May 7, 2017: Oncotarget
https://www.readbyqxmd.com/read/28536532/phase-partitioning-of-gm1-and-its-bodipy-labeled-analog-determine-their-different-binding-to-cholera-toxin
#3
Sami Rissanen, Michal Grzybek, Adam Orłowski, Tomasz Róg, Oana Cramariuc, Ilya Levental, Christian Eggeling, Erdinc Sezgin, Ilpo Vattulainen
Driven by interactions between lipids and proteins, biological membranes display lateral heterogeneity that manifests itself in a mosaic of liquid-ordered (Lo) or raft, and liquid-disordered (Ld) or non-raft domains with a wide range of different properties and compositions. In giant plasma membrane vesicles and giant unilamellar vesicles, specific binding of Cholera Toxin (CTxB) to GM1 glycolipids is a commonly used strategy to label raft domains or Lo membrane environments. However, these studies often use acyl-chain labeled bodipy-GM1 (bdGM1), whose headgroup accessibility and membrane order or phase partitioning may differ from those of GM1, rendering the interpretation of CTxB binding data quite problematic...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/28532689/lipid-composition-of-microdomains-is-altered-in-neuronopathic-gaucher-disease-sheep-brain-and-spleen
#4
Leanne K Hein, Tina Rozaklis, Melissa K Adams, John J Hopwood, Litsa Karageorgos
Gaucher disease is a lysosomal storage disorder caused by a deficiency in glucocerebrosidase activity that leads to accumulation of glucosylceramide and glucosylsphingosine. Membrane raft microdomains are discrete, highly organized microdomains with a unique lipid composition that provide the necessary environment for specific protein-lipid and protein-protein interactions to take place. In this study we purified detergent resistant membranes (DRM; membrane rafts) from the occipital cortex and spleen from sheep affected with acute neuronopathic Gaucher disease and wild-type controls...
May 17, 2017: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/28528321/omega-3-fatty-acids-lipids-and-apoe-lipidation-in-alzheimer-s-disease-a-rationale-for-multi-nutrient-dementia-prevention
#5
Marcus O Grimm, Daniel Michaelson, Tobias Hartmann
In the last decade it has become obvious that Alzheimer's disease (AD) is closely linked to changes in lipids or lipid metabolism. One of the main pathological hallmarks of AD is amyloid-β (Aβ) deposition. Aβ is derived from sequential proteolytic processing of the amyloid precursor protein (APP). Interestingly, both, the APP and all APP secretases are transmembrane proteins which cleave APP close to and in the lipid bilayer. Moreover, apolipoprotein E4 (apoE4) has been identified as the most prevalent genetic risk factor for AD...
May 20, 2017: Journal of Lipid Research
https://www.readbyqxmd.com/read/28524176/lentivirus-mediated-microrna-124-gene-modified-bone-marrow-mesenchymal-stem-cell-transplantation-promotes-the-repair-of-spinal-cord-injury-in-rats
#6
Jia-Lin Song, Wei Zheng, Wei Chen, Yun Qian, Yuan-Ming Ouyang, Cun-Yi Fan
Our study aims to explore the effects of lentivirus-mediated microRNA-124 (miR-124) gene-modified bone marrow mesenchymal stem cell (BMSC) transplantation on the repair of spinal cord injury (SCI) in rats. BMSCs were isolated from the bone marrow of rats. The target gene miR-124 was identified using a luciferase-reporter gene assay. Seventy-two rats were selected for construction of the SCI model, and the rats were randomly divided into the blank group, sham group, SCI group, negative control (NC) group, overexpressed miR-124 group and si-PDXK group...
May 19, 2017: Experimental & Molecular Medicine
https://www.readbyqxmd.com/read/28523231/association-of-anti-gangliosides-antibodies-and-anti-cmv-antibodies-in-guillain-barr%C3%A3-syndrome
#7
Lijuan Wang, Chunqing Shao, Chunjiao Yang, Xixiong Kang, Guojun Zhang
INTRODUCTION: Numerous types of infection were closely related to GBS, mainly including Campylobacter jejuni, Cytomegalovirus, which may lead to the production of anti-gangliosides antibodies (AGA). Currently, although there are increased studies on the AGA and a few studies of anti-CMV antibodies in GBS, the association between them remains poorly documented. Therefore, our research aims to analyze the correlation of anti-CMV antibodies and AGA in GBS. METHODS: A total of 29 patients with GBS were enrolled in this study...
May 2017: Brain and Behavior
https://www.readbyqxmd.com/read/28521265/optimizing-the-electrodiagnostic-accuracy-in-guillain-barr%C3%A3-syndrome-subtypes-criteria-sets-and-sparse-linear-discriminant-analysis
#8
Antonino Uncini, Luigi Ippoliti, Nortina Shahrizaila, Yukari Sekiguchi, Satoshi Kuwabara
OBJECTIVE: To optimize the electrodiagnosis of Guillain-Barré syndrome (GBS) subtypes at first study. METHODS: The reference electrodiagnosis was obtained in 53 demyelinating and 45 axonal GBS patients on the basis of two serial studies and results of anti-ganglioside antibodies assay. We retrospectively employed sparse linear discriminant analysis (LDA), two existing electrodiagnostic criteria sets (Hadden et al., 1998; Rajabally et al., 2015) and one we propose that additionally evaluates duration of motor responses, sural sparing pattern and defines reversible conduction failure (RCF) in motor and sensory nerves at second study...
April 20, 2017: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/28502484/pathogenic-a%C3%AE-a2v-versus-protective-a%C3%AE-a2t-mutation-early-stage-aggregation-and-membrane-interaction
#9
Laura Colombo, Alessio Gamba, Laura Cantù, Mario Salmona, Fabrizio Tagliavini, Valeria Rondelli, Elena Del Favero, Paola Brocca
We investigated the effects of punctual A-to-V and A-to-T mutations in the amyloid precursor protein APP, corresponding to position 2 of Aβ1-42. Those mutations had opposite effects on the onset and progression of Alzheimer disease, the former inducing early AD pathology and the latter protecting against the onset of the disease. We applied Static and Dynamic Light Scattering and Circular Dichroism, to study the different mutants in the early stages of the aggregation process, essential for the disease. Comparative results showed that the aggregation pathways differ in the kinetics and extent of the process, in the size of the aggregates and in the evolution of the secondary structure, resulting in fibrils of different morphology, as seen by AFM...
May 6, 2017: Biophysical Chemistry
https://www.readbyqxmd.com/read/28500248/the-association-of-the-lipidomic-profile-with-features-of-polycystic-ovary-syndrome
#10
Lisa Moran, Piyushkumar Mundra, Helena Teede, Peter J Miekle
Polycystic ovary syndrome (PCOS) affects up to 18% of reproductive-aged women with reproductive and metabolic complications. While lipidomics can identify associations between lipid species and metabolic diseases, no research has examined the association of lipid species with the pathophysiological features of PCOS. The aim of this study was to examine the lipidomic profile in women with and without PCOS. This study was a cross-sectional study in 156 age-matched pre-menopausal women (18-45 years, BMI>25 kg/m2; n=92 with PCOS, n=64 without PCOS)...
May 12, 2017: Journal of Molecular Endocrinology
https://www.readbyqxmd.com/read/28499815/new-insight-into-the-interaction-of-traf2-c-terminal-domain-with-lipid-raft-microdomains
#11
Arianna Ceccarelli, Almerinda Di Venere, Eleonora Nicolai, Anastasia De Luca, Nicola Rosato, Enrico Gratton, Giampiero Mei, Anna Maria Caccuri
In this study we provide the first evidence of the interaction of a truncated-TRAF2 with lipid raft microdomains. We have analyzed this interaction by measuring the diffusion coefficient of the protein in large and giant unilamellar vesicles (LUVs and GUVs, respectively) obtained both from synthetic lipid mixtures and from natural extracts. Steady-state fluorescence measurements performed with synthetic vesicles indicate that this truncated form of TRAF2 displays a tighter binding to raft-like LUVs with respect to the control (POPC-containing LUVs), and that this process depends on the protein oligomeric state...
May 9, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28499578/building-a-biomimetic-membrane-for-neutron-reflectivity-investigation-complexity-asymmetry-and-contrast
#12
Valeria Rondelli, Paola Brocca, Nicola Tranquilli, Giovanna Fragneto, Elena Del Favero, Laura Cantù
The preparation and investigation of model membranes is deserving growing interest both for the physics of complex systems, and for biology. The need of simplified models should preserve mimicking the qualifying characteristics of biological membranes, and keep non-invasive and detailed description. As a main feature, biological membranes are non-homogeneous in the disposition of components, both in the lateral and in the transverse direction. We prepared asymmetric supported membranes containing GM1 ganglioside in biomimetic proportion according to different protocols...
April 29, 2017: Biophysical Chemistry
https://www.readbyqxmd.com/read/28497346/enhanced-autophagy-contributes-to-protective-effects-of-gm1-ganglioside-against-a%C3%AE-1-42-induced-neurotoxicity-and-cognitive-deficits
#13
Ruwei Dai, Shijie Zhang, Wenjun Duan, Renrong Wei, Huifang Chen, Weibin Cai, Lei Yang, Qi Wang
Alzheimer's disease (AD) is a progressive neurodegenerative disorder. The aggregation of Aβ peptides, Aβ1-42 in particular, is thought to be a fundamental pathogenic mechanism leading to the neuronal damage in AD. Recently, monosialoganglioside GM1 is reported to possess pivotal neuroprotection in neurodegenerative diseases. Previous studies have focused on the conformational dynamics and the biochemical interaction of the amyloid-peptide with the GM1 ganglioside, as well as the protective effect of GM1 on cognition...
May 12, 2017: Neurochemical Research
https://www.readbyqxmd.com/read/28495047/similar-clinical-pathological-and-genetic-features-in-chinese-patients-with-autosomal-recessive-and-dominant-charcot-marie-tooth-disease-type-2k
#14
Jun Fu, Shixu Dai, Yuanyuan Lu, Rui Wu, Zhaoxia Wang, Yun Yuan, He Lv
Mutations in the ganglioside-induced differentiation-associated protein 1 gene (GDAP1) cause rare subtypes of Charcot-Marie-Tooth disease (CMT2K and CMT4A). CMT2K is an axonal neuropathy while CMT4A is a demyelinating type. In a series of 169 Chinese CMT patients (79 CMT1, 52 CMT2 and 38 unclassified), four unrelated patients (2.37%) were identified with GDAP1 mutations, including two with autosomal recessive CMT2K (AR-CMT2K) and two dominant CMT2K (AD-CMT2K). All patients had disease onset before 5 years of age, and presented with muscle weakness, atrophy, and mild sensory disturbance in distal limbs...
April 20, 2017: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/28491873/b-cd8-t-cell-interactions-in-the-anti-idiotypic-response-against-a-self-antibody
#15
Darel Martínez, Amaury Pupo, Lianet Cabrera, Judith Raymond, Nichol E Holodick, Ana María Hernández
P3 is a murine, germline, IgM mAb that recognizes N-glycolylated gangliosides and other self-antigens. This antibody is able to induce an anti-idiotypic IgG response and B-T idiotypic cascade, even in the absence of any adjuvant or carrier protein. P3 mAb immunization induces the expression of activation markers in a significant percentage of B-1a cells in vivo. Interestingly, transfer of both B-1a and B-2 to BALB/Xid mice was required to recover anti-P3 IgG response in this model. In fact, P3 mAb activated B-2 cells, in vitro, inducing secretion of IFN-γ and IL-4, although this activation was not detected ex vivo...
2017: Journal of Immunology Research
https://www.readbyqxmd.com/read/28470148/peroxisomal-dysfunctions-cause-lysosomal-storage-and-axonal-kv1-channel-redistribution-in-peripheral-neuropathy
#16
Sandra Kleinecke, Sarah Richert, Livia de Hoz, Britta Brügger, Theresa Kungl, Ebrahim Asadollahi, Susanne Quintes, Judith Blanz, Rhona McGonigal, Kobra Naseri, Michael W Sereda, Timo Sachsenheimer, Christian Lüchtenborg, Wiebke Möbius, Hugh Willison, Myriam Baes, Klaus-Armin Nave, Celia Michèle Kassmann
Impairment of peripheral nerve function is frequent in neurometabolic diseases, but mechanistically not well understood. Here, we report a novel disease mechanism and the finding that glial lipid metabolism is critical for axon function, independent of myelin itself. Surprisingly, nerves of Schwann cell-specific Pex5 mutant mice were unaltered regarding axon numbers, axonal calibers, and myelin sheath thickness by electron microscopy. In search for a molecular mechanism, we revealed enhanced abundance and internodal expression of axonal membrane proteins normally restricted to juxtaparanodal lipid-rafts...
May 4, 2017: ELife
https://www.readbyqxmd.com/read/28463983/imaging-mass-spectrometry-identifies-prognostic-ganglioside-species-in-rodent-intracranial-transplants-of-glioma-and-medulloblastoma
#17
Leonardo Ermini, Elena Morganti, Alexander Post, Behzad Yeganeh, Isabella Caniggia, Michael Leadley, Claudia C Faria, James T Rutka, Martin Post
Matrix-assisted laser desorption ionization (MALDI) imaging mass spectrometry (MALDI-MSI) allows us to investigate the distribution of lipid molecules within tissues. We used MALDI-MSI to identify prognostic gangliosides in tissue sections of rat intracranial allografts of rat glioma and mouse intracranial xenografts of human medulloblastoma. In the healthy adult rodent brain, GM1 and GD1 were the main types of glycolipids. Both gangliosides were absent in both intracranial transplants. The ganglioside GM3 was not present in the healthy adult brain but was highly expressed in rat glioma allografts...
2017: PloS One
https://www.readbyqxmd.com/read/28451919/a-novel-conditional-sgsh-knockout-mouse-model-recapitulates-phenotypic-and-neuropathic-deficits-of-sanfilippo-syndrome
#18
Adeline A Lau, Barbara M King, Carly L Thorsen, Sofia Hassiotis, Helen Beard, Paul J Trim, Lauren S Whyte, Sarah J Tamang, Stephen K Duplock, Marten F Snel, John J Hopwood, Kim M Hemsley
Mucopolysaccharidosis (MPS) type IIIA, or Sanfilippo syndrome, is a neurodegenerative lysosomal storage disorder caused by a deficiency of the lysosomal enzyme N-sulfoglucosamine sulfohydrolase (SGSH), involved in the catabolism of heparan sulfate. The clinical spectrum is broad and the age of symptom onset and the degree of preservation of cognitive and motor functions appears greatly influenced by genotype. To explore this further, we generated a conditional knockout (Sgsh (KO) ) mouse model with ubiquitous Sgsh deletion, and compared the clinical and pathological phenotype with that of the spontaneous Sgsh (D31N) MPS-IIIA mouse model...
April 27, 2017: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/28450686/serial-neurophysiological-and-neurophysiological-examinations-for-delayed-facial-nerve-palsy-in-a-patient-with-fisher-syndrome
#19
Motoyuki Umekawa, Keiko Hatano, Hideyuki Matsumoto, Takahiro Shimizu, Hideji Hashida
The patient was a 47-year-old man who presented with diplopia and gait instability with a gradual onset over the course of three days. Neurological examinations showed ophthalmoplegia, diminished tendon reflexes, and truncal ataxia. Tests for anti-GQ1b antibodies and several other antibodies to ganglioside complex were positive. We made a diagnosis of Fisher syndrome. After administration of intravenous immunoglobulin, the patient's symptoms gradually improved. However, bilateral facial palsy appeared during the recovery phase...
April 27, 2017: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/28444683/-atypical-guillain-barre-syndrome-clustering-is-it-necessary-to-reconsider-the-diagnostic-criteria-and-microbiological-protocol
#20
A Dominguez-Mayoral, C Gutierrez, J M Lopez-Dominguez, S Eichau, J Abril, G Navarro-Mascarell, M A Quesada-Garcia, M Ramos, M Alvarez-Lopez, C Menendez-De Leon, G Izquierdo
INTRODUCTION: Guillain-Barre syndrome is classically defined as a symmetrical ascending acute polyradiculoneuropathy, although there are atypical variants that make diagnosis difficult. CASE REPORTS: The medical data of six patients in our hospital area are collected during the first quarter of 2013. Lumbar punctures, imaging, neurophysiological studies, ganglioside antibodies and serologies have been proposed in all cases. We focus on the atypical features as late hyporeflexia, increased frequency of asymmetry and distal paresis and initial fever...
May 1, 2017: Revista de Neurologia
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