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https://www.readbyqxmd.com/read/28714776/tissue-localization-of-glycosphingolipid-accumulation-in-a-gaucher-disease-mouse-brain-by-lc-esi-ms-ms-and-high-resolution-maldi-imaging-mass-spectrometry
#1
E Ellen Jones, Wujuan Zhang, Xueheng Zhao, Cristine Quiason, Stephanie Dale, Sheerin Shahidi-Latham, Gregory A Grabowski, Kenneth D R Setchell, Richard R Drake, Ying Sun
To better understand regional brain glycosphingolipid (GSL) accumulation in Gaucher disease (GD) and its relationship to neuropathology, a feasibility study using mass spectrometry and immunohistochemistry was conducted using brains derived from a GD mouse model (4L/PS/NA) homozygous for a mutant GCase (V394L [4L]) and expressing a prosaposin hypomorphic (PS-NA) transgene. Whole brains from GD and control animals were collected using one hemisphere for MALDI FTICR IMS analysis and the other for quantitation by LC-ESI-MS/MS...
July 1, 2017: SLAS Discovery
https://www.readbyqxmd.com/read/28708322/ganglioside-gm3-suppresses-lipopolysaccharide-induced-inflammatory-responses-in-raw-264-7-macrophage-cells-through-nf-%C3%AE%C2%BAb-ap-1-and-mapks-signaling
#2
Junyoung Park, Choong-Hwan Kwak, Sun-Hyung Ha, Kyung-Min Kwon, Fukushi Abekura, Seung-Hak Cho, Young-Chae Chang, Young-Choon Lee, Ki-Tae Ha, Tae-Wook Chung, Cheorl-Ho Kim
Gangliosides are known to specifically inhibit vascular leukocyte recruitment and consequent interaction with the injured endothelium, the basic inflammatory process. In this study, we have found that the production of nitric oxide (NO), a main regulator of inflammation, is suppressed by GM3 on murine macrophage RAW264.7 cells, when induced by LPS. In addition, GM3 attenuated the increase in cyclooxyenase-2 (COX-2) protein and mRNA levels in lipopolysaccharide (LPS)-activated RAW 264.7 cells in a dose-dependent manner...
July 14, 2017: Journal of Cellular Biochemistry
https://www.readbyqxmd.com/read/28701774/inhibition-of-%C3%AE-glucocerebrosidase-activity-preserves-motor-unit-integrity-in-a-mouse-model-of-amyotrophic-lateral-sclerosis
#3
Alexandre Henriques, Mylene Huebecker, Hélène Blasco, Céline Keime, Christian R Andres, Philippe Corcia, David A Priestman, Frances M Platt, Michael Spedding, Jean-Philippe Loeffler
Recent metabolomic reports connect dysregulation of glycosphingolipids, particularly ceramide and glucosylceramide, to neurodegeneration and to motor unit dismantling in amyotrophic lateral sclerosis at late disease stage. We report here altered levels of gangliosides in the cerebrospinal fluid of amyotrophic lateral sclerosis patients in early disease stage. Conduritol B epoxide is an inhibitor of acid beta-glucosidase, and lowers glucosylceramide degradation. Glucosylceramide is the precursor for all of the more complex glycosphingolipids...
July 12, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28701394/single-particle-detection-of-transcription-following-rotavirus-entry
#4
Eric N Salgado, Srigokul Upadhyayula, Stephen C Harrison
Infectious rotavirus particles are triple-layered, icosahedral assemblies. The outer layer proteins, VP4 (cleaved to VP8* and VP5*) and VP7, surround a transcriptionally competent, double-layer particle (DLP), which they deliver into the cytosol. During entry of rhesus rotavirus, VP8* interacts with cell-surface gangliosides, allowing engulfment into a membrane vesicle by a clathrin-independent process. Escape into the cytosol and outer-layer shedding depend on interaction of a hydrophobic surface on VP5* with the membrane bilayer and on a large-scale conformational change...
July 12, 2017: Journal of Virology
https://www.readbyqxmd.com/read/28698248/ganglioside-glycosyltransferases-are-s-acylated-at-conserved-cysteine-residues-involved-in-homodimerization
#5
Sabrina Chumpen Ramirez, Fernando M Ruggiero, Jose Luis Daniotti, Javier Valdez Taubas
Ganglioside glycosyltransferases (GGTs) are type-II membrane proteins bearing a short N-terminal cytoplasmic tail, a transmembrane domain (TMD), and a lumenal catalytic domain. The expression and activity of these enzymes largely determine the quality of the glycolipids that decorate mammalian cells membranes. Many glycosyltransferases are themselves glycosylated and this is important for their proper localisation, but few if any other post-translational modifications of these proteins have been reported. Here we show that the GGTs, ST3Gal-V, ST8Sia-I, and β4GalNAcT-I are S-acylated at conserved cysteine residues located close the cytoplasmic border of their TMDs...
July 11, 2017: Biochemical Journal
https://www.readbyqxmd.com/read/28692598/membrane-lipids-and-cell-signaling
#6
Hannah Sunshine, Maria Luisa Iruela-Arispe
PURPOSE OF REVIEW: Reception and transmission of signals across the plasma membrane has been a function generally attributed to transmembrane proteins. In the last 3 years, however, a growing number of reports have further acknowledged important contributions played by membrane lipids in the process of signal transduction. RECENT FINDINGS: In particular, the constituency of membrane lipids can regulate how proteins with SH2 domains and molecules like K-Ras expose their catalytic domains to the cytosol and interact with effectors and second messengers...
July 7, 2017: Current Opinion in Lipidology
https://www.readbyqxmd.com/read/28687233/overview-of-immune-abnormalities-in-lysosomal-storage-disorders
#7
REVIEW
Donato Rigante, Clelia Cipolla, Umberto Basile, Francesca Gulli, Maria Cristina Savastano
The critical relevance of the lysosomal compartment for normal cellular function can be proved by numbering the clinical phenotypes that arise in lysosomal storage disorders (LSDs), a group of around 70 different monogenic autosomal or X-linked syndromes, caused by specific lysosomal enzyme deficiencies: all LSDs are characterized by progressive accumulation of heterogeneous biologic materials in the lysosomes of various parts of the body such as viscera, skeleton, skin, heart, and central nervous system. At least a fraction of LSDs has been associated with mixed abnormalities involving the immune system, while some patients with LSDs may result more prone to autoimmune phenomena...
July 4, 2017: Immunology Letters
https://www.readbyqxmd.com/read/28686720/free-cholesterol-and-cholesterol-esters-in-bovine-oocytes-implications-in-survival-and-membrane-raft-organization-after-cryopreservation
#8
Jorgelina Buschiazzo, Glenda L Ríos, Jesica R Canizo, Silvia S Antollini, Ricardo H Alberio
Part of the damage caused by cryopreservation of mammalian oocytes occurs at the plasma membrane. The addition of cholesterol to cell membranes as a strategy to make it more tolerant to cryopreservation has been little addressed in oocytes. In order to increase the survival of bovine oocytes after cryopreservation, we proposed not only to increase cholesterol level of oocyte membranes before vitrification but also to remove the added cholesterol after warming, thus recovering its original level. Results from our study showed that modulation of membrane cholesterol by methyl-β-cyclodextrin (MβCD) did not affect the apoptotic status of oocytes and improved viability after vitrification yielding levels of apoptosis closer to those of fresh oocytes...
2017: PloS One
https://www.readbyqxmd.com/read/28678088/treatment-of-transient-peripheral-neuropathy-during-chimeric-14-18-antibody-therapy-in-children-with-neuroblastoma-a-case-series
#9
Pranathi Ari, Michelle Kars, Holly Meany, Sophie Pestieau
Children with high-risk neuroblastoma are currently treated with a chimeric monoclonal antibody against GD2 ganglioside (chimeric 14.18). The treatment improves survival but causes transient neuropathic pain-like syndrome. We retrospectively studied 16 children with neuroblastoma receiving GD2 therapy. To manage pain, all patients received morphine via nurse-controlled analgesia or patient-controlled analgesia. Mean daily pain scores ranged from 0 to 5 and all children had a 0 pain score upon discharge. No major side effects were noted, suggesting morphine via nurse-controlled analgesia/patient-controlled analgesia is effective in controlling transient neuropathic pain in children receiving GD2 antibody therapy...
July 3, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28673733/enhanced-soluble-production-of-cholera-toxin-b-subunit-in-escherichia-coli-by-co-expression-of-skp-chaperones
#10
Yuanpeng Zhang, Xuwen Qiao, Xiaoming Yu, Jin Chen, Liting Hou, Zhixiang Bi, Qisheng Zheng, Jibo Hou
The cholera toxin B subunit (CTB) is a nontoxic portion of the cholera toxin that retains mucosal adjuvant properties. Expression of CTB in Escherichia coli is difficult as CTB aggregates and accumulates as insoluble inclusion bodies. To remedy this problem, the periplasmic chaperone, SKP, was investigated as possible co-expression partner to increase the solubility of recombinant CTB (rCTB) in E. coli. The result showed co-expression of SKP enhanced the soluble expression of rCTB in E. coli. Moreover, soluble rCTB was successfully expressed and secreted into the periplasmic space through the direction of the LTB leader signal...
June 30, 2017: Protein Expression and Purification
https://www.readbyqxmd.com/read/28673555/brain-involvement-in-charcot-marie-tooth-disease-due-to-ganglioside-induced-differentiation-associated-protein-1-mutation
#11
Fouad Al-Ghamdi, Irina Anselm, Edward Yang, Partha S Ghosh
Charcot-Marie-Tooth (CMT) due to ganglioside-induced differentiation associated-protein 1 (GDAP1) gene mutation can be inherited as an autosomal recessive (severe phenotype) or dominant (milder phenotype) disorder. GDAP1 protein, located in the outer mitochondrial membrane, is involved in the mitochondrial fission. Brain imaging abnormalities have not been reported in this condition. We described an 8-year-old boy who had an early onset autosomal recessive neuropathy. Whole exome sequencing revealed compound heterozygous mutations in the GDAP1 gene: c...
June 7, 2017: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/28667538/detection-of-naturally-occurring-human-antibodies-against-gangliosides-by-elisa
#12
Ana María Hernández, Nely Rodríguez-Zhurbenko
Gangliosides are sialic acid-containing glycolipids that have been considered attractive targets for cancer immunotherapy, based on the qualitative and quantitative changes they suffer during malignant transformation and due to their importance for tumor biology. Natural antibodies against gangliosides have been detected not only in cancer patients but also in healthy donors. The presence of these antibodies can be used as diagnostic or prognostic factor. However, these responses are difficult to detect because anti-ganglioside antibodies are usually of IgM isotype and low affinity...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28666962/decreased-calcium-flux-in-niemann-pick-type-c1-patient-specific-ipsc-derived-neurons-due-to-higher-amount-of-calcium-impermeable-ampa-receptors
#13
Michael Rabenstein, Franziska Peter, Sarah Joost, Michaela Trilck, Arndt Rolfs, Moritz J Frech
Niemann-Pick disease type C1 (NPC1) is a rare progressive neurodegenerative disorder caused by mutations in the NPC1 gene, resulting mainly in the accumulation of cholesterol and the ganglioside GM2. Recently, we described accumulations of these lipids in neuronal differentiated cells derived from NPC1 patient-specific induced pluripotent stem cells (iPSCs). As these lipids are essential for proper cell membrane composition, we were interested in the expression and function of voltage-gated ion channels and excitatory AMPA receptors (AMPARs) in neurons derived from three patient-specific iPSC lines...
June 27, 2017: Molecular and Cellular Neurosciences
https://www.readbyqxmd.com/read/28660589/on-electrochemical-methods-for-determination-of-protein-lipid-interaction
#14
Zhiping Hu, Yanli Mao
Amyloid-β (Aβ) peptides are important and reliable molecular biomarkers for the diagnosis and prognosis of Alzheimer's disease. Aggregation and fibrillization of Aβ peptides on ganglioside GM1 (GM1)-containing lipid membranes is considered a cause of neurodegenerative disease. Because GM1 is abundant in the central nervous system and plays a key role in the aggregation of Aβ, the interaction of Aβ with supported planar lipid bilayers (SPBs) containing GM1 is of great significance. We have prepared SPBs containing GM1 in order to study the electrochemical characteristics of GM1/sphingomyelin/cholesterol SPBs and their interaction with Aβ(1-40) by cyclic voltammetry and electrochemical impedance spectroscopy (EIS), which proves that electrochemical is a promising method for analyzing the interaction between peptides and lipid membranes...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28656586/psa-ncam-expression-in-the-teleost-optic-tectum-is-related-to-ecological-niche-and-use-of-vision-in-finding-food
#15
I Labak, V Pavić, M Zjalić, S Blažetić, B Viljetić, E Merdić, M Heffer
In this study, tangential migration and neuronal connectivity organization were analysed in the optic tectum of seven different teleosts through the expression of polysialylated neural cell adhesion molecule (PSA-NCAM) in response to ecological niche and use of vision. Reduced PSA-NCAM expression in rainbow trout Oncorhynchus mykiss optic tectum occurred in efferent layers, while in pike Esox lucius and zebrafish Danio rerio it occurred in afferent and efferent layers. Zander Sander lucioperca and European eel Anguilla anguilla had very low PSA-NCAM expression in all tectal layers except in the stratum marginale...
June 27, 2017: Journal of Fish Biology
https://www.readbyqxmd.com/read/28651685/erratum-to-ganglioside-gm1-influences-the-proliferation-rate-of-mouse-induced-pluripotent-stem-cells
#16
Jae-Sung Ryu, Kyu-Tae Chang, Ju-Taek Lee, Malg-Um Lim, Hyun-Ki Min, Yoon-Ju Na, Su-Bin Lee, Gislain Moussavou, Sun-Uk Kim, Ji-Su Kim, Kinarm Ko, Kisung Ko, Kyung-A Hwang, Eun-Jeong Jeong, Jeong-Woong Lee, Young-Kug Choo
The BMB Reports would like to correct in the ACKNOWLEDGEMENTS of BMB Rep. 45(12), 713-718 titled "Ganglioside GM1 influences the proliferation rate of mouse induced pluripotent stem cells".
June 2017: BMB Reports
https://www.readbyqxmd.com/read/28645851/nanotubes-connecting-b-lymphocytes-high-impact-of-differentiation-dependent-lipid-composition-on-their-growth-and-mechanics
#17
Eszter A Tóth, Ádám Oszvald, Mária Péter, Gábor Balogh, Anikó Osteikoetxea-Molnár, Tamás Bozó, Edina Szabó-Meleg, Miklós Nyitrai, Imre Derényi, Miklós Kellermayer, Toshiyuki Yamaji, Kentaro Hanada, László Vígh, János Matkó
Nanotubes (NTs) are thin, long membranous structures forming novel, yet poorly known communication pathways between various cell types. Key mechanisms controlling their growth still remained poorly understood. Since NT-forming capacity of immature and mature B cells was found largely different, we investigated how lipid composition and molecular order of the membrane affect NT-formation. Screening B cell lines with various differentiation stages revealed that NT-growth linearly correlates with membrane ganglioside levels, while it shows maximum as a function of cholesterol level...
June 20, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28643517/strategy-for-comprehensive-profiling-and-identification-of-acidic-glycosphingolipids-using-ultra-high-performance-liquid-chromatography-coupled-with-quadrupole-time-of-flight-mass-spectrometry
#18
Ting Hu, Zhixin Jia, Jin-Lan Zhang
Acidic glycosphingolipids (AGSLs), which mainly consist of ganglioside and sulfatide moieties, are highly concentrated in the central nervous system. Comprehensive profiling of AGSLs has historically been challenging because of their high complexity and the lack of standards. In this study, a novel strategy was developed to comprehensively profile AGSLs using ultra-high-performance liquid chromatography-quadrupole time-of-flight mass spectrometry. Ganglioside isomers with different glycan chains such as GD1a/GD1b were completely separated on a C18 column for the first time to our knowledge, facilitated by the addition of formic acid in the mobile phase...
July 7, 2017: Analytical Chemistry
https://www.readbyqxmd.com/read/28608355/-neuronal-glycolipids-regulate-glial-cell-division-negatively-during-development-and-following-a-lesion
#19
REVIEW
M Nieto-Sampedro, V C Muneton-Gomez
Glial cells in the central nervous system of adult mammals outnumber neurons 10-fold. Their number remains stationary throughout adulthood, controlled by the concomitant presence of mitogens and mitogen inhibitors. The most abundant inhibitor, neurostatin, is ganglioside GD1b O-acetylated on hydroxyl 9 of its outermost sialic acid. Neurostatin inhibited the proliferation of primary microglia and astroblasts in culture (cytostatic) as well as both rodent and human glioma cells (cytotoxic) at nanomolar concentrations...
June 16, 2017: Revista de Neurologia
https://www.readbyqxmd.com/read/28604556/immunologic-response-elicited-in-breast-cancer-patients-receiving-a-neugcgm3-based-vaccine-as-adjuvant-therapy
#20
Anet Valdes-Zayas, Zuyen Gonzalez, Vladimir Mulens, Aliz M Vega, Kirenia Perez, Patricia Lorenzo-Luaces, Maria C Rubio, Anabely Estevez, Idelmis Curbelo, Luis E Fernandez, Tania Crombet, Zaima Mazorra
This study aimed to investigate the immunogenicity of a cancer vaccine consisting of the NeuGcGM3 ganglioside combined with the outer membrane protein complex of Neisseria meningitides to form very small size particles. The vaccine is administered together with Montanide ISA51, as adjuvant treatment for breast cancer patients. After surgical resection and standard first-line chemo/radiotherapy, breast cancer patients in stage II-III were enrolled in a phase III clinical trial and allocated into 2 strata, according to the number of positive lymph nodes [stratum I (0-3); stratum II (≥4)]...
June 9, 2017: Journal of Immunotherapy
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