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https://www.readbyqxmd.com/read/29223996/metabolic-myopathies-a-practical-approach
#1
REVIEW
James B Lilleker, Yann Shern Keh, Federico Roncaroli, Reena Sharma, Mark Roberts
Metabolic myopathies are a diverse group of rare genetic disorders and their associated muscle symptoms may be subtle. Patients may present with indolent myopathic features, exercise intolerance or recurrent rhabdomyolysis. Diagnostic delays are common and clinicians need a high index of suspicion to recognise and differentiate metabolic myopathies from other conditions that present in a similar fashion. Standard laboratory tests may be normal or non-specific, particularly between symptomatic episodes. Targeted enzyme activity measurement and next-generation genetic sequencing are increasingly used...
December 9, 2017: Practical Neurology
https://www.readbyqxmd.com/read/29216826/6s-rna-plays-a-role-in-recovery-from-nitrogen-depletion-in-synechocystis-sp-pcc-6803
#2
Beate Heilmann, Kaisa Hakkila, Jens Georg, Taina Tyystjärvi, Wolfgang R Hess, Ilka M Axmann, Dennis Dienst
BACKGROUND: The 6S RNA is a global transcriptional riboregulator, which is exceptionally widespread among most bacterial phyla. While its role is well-characterized in some heterotrophic bacteria, we subjected a cyanobacterial homolog to functional analysis, thereby extending the scope of 6S RNA action to the special challenges of photoautotrophic lifestyles. RESULTS: Physiological characterization of a 6S RNA deletion strain (ΔssaA) demonstrates a delay in the recovery from nitrogen starvation...
December 8, 2017: BMC Microbiology
https://www.readbyqxmd.com/read/29215735/vacuolated-pas-positive-lymphocytes-as-an-hallmark-of-pompe-disease-and-other-myopathies-related-to-impaired-autophagy
#3
Angelo Pascarella, Chiara Terracciano, Olimpia Farina, Luca Lombardi, Teresa Esposito, Filomena Napolitano, Giuseppina Franzese, Giovanni Panella, Francesco Tuccillo, Giancarlo la Marca, Sergio Bernardini, Silvia Boffo, Antonio Giordano, Mariarosa Anna Beatrice Melone, Giuseppe Di Iorio, Simone Sampaolo
Autosomal recessive Pompe disease is a lysosomal disorder caused by mutations of the acid-α-glucosidase (GAA) gene. Deficiency of GAA enzyme leads to glycogen accumulation and autophagy impairment in cardiac and skeletal muscles, but also in lymphocytes. Since an effective therapy is available, a rapid, sensitive and specific test is crucial to early identify affected subjects. Number of lymphocytes containing PAS-positive vacuoles was evaluated on blood films from 72 consecutive adult patients with hyperckemia and/or muscle weakness, 13 genetically confirmed late-onset-Pompe-disease (LOPD) and 13 of their offspring...
December 7, 2017: Journal of Cellular Physiology
https://www.readbyqxmd.com/read/29211918/decellularized-human-placenta-supports-hepatic-tissue-and-allows-rescue-in-acute-liver-failure
#4
Zurab Kakabadze, Anne Kakabadze, David Chakhunashvili, Lika Karalashvili, Ekaterine Berishvili, Yogeshwar Sharma, Sanjeev Gupta
Tissue engineering with scaffolds to form transplantable organs is of wide interest. Decellularized tissues have been tested for this purpose, although supplies of healthy donor tissues, vascular recellularization for perfusion and tissue homeostasis in engineered organs pose challenges. We hypothesized decellularized human placenta will be suitable for tissue engineering. The universal availability and unique structures of placenta for accommodating tissue, including presence of embedded vessels, were major attractions...
December 6, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/29196846/the-availability-of-water-associated-with-glycogen-during-dehydration-a-reservoir-or-raindrop
#5
Roderick F G J King, Ben Jones, John P O'Hara
PURPOSE: This study evaluated whether glycogen-associated water is a protected entity not subject to normal osmotic homeostasis. An investigation into practical and theoretical aspects of the functionality of this water as a determinant of osmolality, dehydration, and glycogen concentration was undertaken. METHODS: In vitro experiments were conducted to determine the intrinsic osmolality of glycogen-potassium phosphate mixtures as would be found intra-cellularly at glycogen concentrations of 2% for muscle and 5 and 10% for liver...
December 1, 2017: European Journal of Applied Physiology
https://www.readbyqxmd.com/read/29187643/rescue-of-pompe-disease-in-mice-by-aav-mediated-liver-delivery-of-secretable-acid-%C3%AE-glucosidase
#6
Francesco Puzzo, Pasqualina Colella, Maria G Biferi, Deeksha Bali, Nicole K Paulk, Patrice Vidal, Fanny Collaud, Marcelo Simon-Sola, Severine Charles, Romain Hardet, Christian Leborgne, Amine Meliani, Mathilde Cohen-Tannoudji, Stephanie Astord, Bernard Gjata, Pauline Sellier, Laetitia van Wittenberghe, Alban Vignaud, Florence Boisgerault, Martine Barkats, Pascal Laforet, Mark A Kay, Dwight D Koeberl, Giuseppe Ronzitti, Federico Mingozzi
Glycogen storage disease type II or Pompe disease is a severe neuromuscular disorder caused by mutations in the lysosomal enzyme, acid α-glucosidase (GAA), which result in pathological accumulation of glycogen throughout the body. Enzyme replacement therapy is available for Pompe disease; however, it has limited efficacy, has high immunogenicity, and fails to correct pathological glycogen accumulation in nervous tissue and skeletal muscle. Using bioinformatics analysis and protein engineering, we developed transgenes encoding GAA that could be expressed and secreted by hepatocytes...
November 29, 2017: Science Translational Medicine
https://www.readbyqxmd.com/read/29156528/actein-ameliorates-hepatic-steatosis-and-fibrosis-in-high-fat-diet-induced-nafld-by-regulation-of-insulin-and-leptin-resistant
#7
Hong-Jun Chen, Jin Liu
Insulin and leptin resistance are highly involved in metabolic syndrome and non-alcoholic fatty liver disease (NAFLD). Presently, no approved treatment is available. Actein is isolated from the rthizomes of Cimicifuga foetida, a triterpene glycoside, exhibiting important biological properties, such as anti-inflammatory, anti-cancer, and anti-oxidant activity. However, its effects on metabolic syndrome are poorly understood. The aims of the study were mainly to investigate the molecular mechanisms regulating insulin and leptin resistance, and lipogenic action of actein in high fat diet-fed mice...
November 15, 2017: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/29152458/lysosomal-storage-diseases
#8
REVIEW
Carlos R Ferreira, William A Gahl
Lysosomes are cytoplasmic organelles that contain a variety of different hydrolases. A genetic deficiency in the enzymatic activity of one of these hydrolases will lead to the accumulation of the material meant for lysosomal degradation. Examples include glycogen in the case of Pompe disease, glycosaminoglycans in the case of the mucopolysaccharidoses, glycoproteins in the cases of the oligosaccharidoses, and sphingolipids in the cases of Niemann-Pick disease types A and B, Gaucher disease, Tay-Sachs disease, Krabbe disease, and metachromatic leukodystrophy...
May 25, 2017: Translational Science of Rare Diseases
https://www.readbyqxmd.com/read/29124014/a-molecular-analysis-of-the-gaa-gene-and-clinical-spectrum-in-38-patients-with-pompe-disease-in-japan
#9
Yasuyuki Fukuhara, Naoko Fuji, Narutoshi Yamazaki, Asami Hirakiyama, Tetsuharu Kamioka, Joo-Hyun Seo, Ryuichi Mashima, Motomichi Kosuga, Torayuki Okuyama
Pompe disease is an autosomal recessive disorder caused by acid α-glucosidase (GAA) deficiency, which results in the accumulation of glycogen in lysosomes in multiple tissues, including cardiac, skeletal, and smooth muscle cells. Thus far, 558 sequence variants of the GAA gene have been published in the Pompe Disease Mutation Database, and some mutations appear with considerable frequency in particular ethnic groups, such as Caucasians, Taiwanese, Chinese, and Koreans. However, the GAA mutation pattern in Japanese patients remains poorly understood...
March 2018: Molecular Genetics and Metabolism Reports
https://www.readbyqxmd.com/read/29106746/low-dose-lithium-regimen-enhances-endochondral-fracture-healing-in-osteoporotic-rodent-bone
#10
Kathak Vachhani, Cari Whyne, Yufa Wang, David Burns, Diane Nam
Osteoporotic bone fractures are highly prevalent and involve lengthy recovery. Lithium, commonly used in psychiatric medicine, inhibits glycogen synthase kinase-3β in the Wnt/β-catenin pathway, leading to up-regulation of osteogenesis. Our recent preclinical work demonstrated that a 20mg/kg lithium dose administered beginning 7 days post-fracture for 14 days optimally improved femoral fracture healing in healthy rats at 4 weeks post fracture (46% higher torsional strength). In this study, lithium treatment was evaluated for healing of osteoporotic bone fractures...
November 6, 2017: Journal of Orthopaedic Research: Official Publication of the Orthopaedic Research Society
https://www.readbyqxmd.com/read/29059712/selectivity-cell-permeability-and-oral-availability-studies-of-novel-bromophenol-derivative-hpn-as-ptp1b-inhibitor
#11
Jiao Luo, Qi Xu, Bo Jiang, Renshuai Zhang, Xiaoling Jia, Xiangqian Li, Lijun Wang, Chuanlong Guo, Ning Wu, Dayong Shi
BACKGROUND AND PURPOSE: Protein tyrosine phosphatase 1B (PTP1B) negatively regulates insulin signaling by tyrosine dephosphorylation of insulin receptor. It is a highly validated target for type 2 diabetes therapeutics. Here, the anti-diabetic effects of HPN were evaluated in the diabetic BKS db mice. EXPERIMENTAL APPROACH: The inhibitory mode of PTP1B was determined according to the Lineweaver-Burk plot in the presence of HPN. Surface plasmon resonance (SPR) assay and molecular docking were used to study the interaction between HPN and PTP1B...
October 23, 2017: British Journal of Pharmacology
https://www.readbyqxmd.com/read/29057245/a-case-report-of-congenital-glycogen-storage-liver-cirrhosis-treated-with-bone-marrow-derived-stem-cells
#12
Terek W Wehbe, Nassim H Abi Chahine, Abdul-Rahman A Annous, Mohammad A Ferri, Robert C Boulous, Majid F El-Mestrah
Liver cirrhosis represents a state of end-stage failure that is usually fatal. The condition results in liver dysfunction, recurrent ascites, encephalopathy, renal failure, splenomegaly, bleeding, and a poor quality of life in general. With the current severe shortage of donated organs, the only available treatment in the developing countries remains palliative care. We report a case of congenital metabolic liver cirrhosis due to glycogen storage disease diagnosed at age eight. The patient, a male, received bone marrow derived mononuclear cells (BMMC) at age 16 and again at age 17 with significant improvement of his biochemical liver function tests, ascites build-up, asthenia, splenomegaly and quality of life...
2017: Stem Cell Investigation
https://www.readbyqxmd.com/read/29056376/toxic-effects-of-multi-walled-carbon-nanotubes-on-bivalves-comparison-between-functionalized-and-nonfunctionalized-nanoparticles
#13
Lucia De Marchi, Victor Neto, Carlo Pretti, Etelvina Figueira, Federica Chiellini, Andrea Morelli, Amadeu M V M Soares, Rosa Freitas
Despite of the large array of available carbon nanotube (CNT) configurations that allow different industrial and scientific applications of these nanoparticles, their impacts on aquatic organisms, especially on invertebrate species, are still limited. To our knowledge, no information is available on how surface chemistry alteration (functionalization) of CNTs may impact the toxicity of these NPs to bivalve species after a chronic exposure. For this reason, the impacts induced by chronic exposure (28days) to unfunctionalized MWCNTs (Nf-MWCNTs) in comparison with functionalized MWCNTs (f-MWCNTs), were evaluated in R...
October 19, 2017: Science of the Total Environment
https://www.readbyqxmd.com/read/28965119/thiadiazolidinone-8-ameliorates-inflammation-associated-with-experimental-colitis-in-mice
#14
Vanessa Mateus, João Rocha, Paula Alves, Hélder Mota-Filipe, Bruno Sepodes, Rui Pinto
Thiadiazolidinone-8 (TDZD-8) is an effective thiadiazolidinone derivate that is able to suppress the expression of inflammatory cytokines; it also presents tissue protective actions by glycogen synthase kinase (GSK)-3β inhibition, promoting thus an anti-inflammatory effect. Since inflammatory bowel disease is a chronic disease with reduced quality of life, where currently available therapies are only able to induce or maintain the patient in remission, it is crucial to investigate new pharmacological approaches...
September 30, 2017: Pharmacology
https://www.readbyqxmd.com/read/28946905/metabolic-adjustment-upon-repetitive-substrate-perturbations-using-dynamic-13-c-tracing-in-yeast
#15
C A Suarez-Mendez, C Ras, S A Wahl
BACKGROUND: Natural and industrial environments are dynamic with respect to substrate availability and other conditions like temperature and pH. Especially, metabolism is strongly affected by changes in the extracellular space. Here we study the dynamic flux of central carbon metabolism and storage carbohydrate metabolism under dynamic feast/famine conditions in Saccharomyces cerevisiae. RESULTS: The metabolic flux reacts fast and sensitive to cyclic perturbations in substrate availability...
September 25, 2017: Microbial Cell Factories
https://www.readbyqxmd.com/read/28941596/robust-suppression-of-cardiac-energy-catabolism-with-marked-accumulation-of-energy-substrates-during-lipopolysaccharide-induced-cardiac-dysfunction-in-mice
#16
Yogi Umbarawan, Mas Rizky A A Syamsunarno, Hideru Obinata, Aiko Yamaguchi, Hiroaki Sunaga, Hiroki Matsui, Takako Hishiki, Tomomi Matsuura, Norimichi Koitabashi, Masaru Obokata, Hirofumi Hanaoka, Anwarul Haque, Fumio Kunimoto, Yoshito Tsushima, Makoto Suematsu, Masahiko Kurabayashi, Tatsuya Iso
BACKGROUND: Myocardial contractile dysfunction in sepsis has been attributed mainly to increased inflammatory cytokines, insulin resistance, and impaired oxidative phosphorylation of fatty acids (FAs). However, precise molecular mechanisms underlying the cardiac dysfunction in sepsis remain to be determined. We previously reported major shift in myocardial energy substrates from FAs to glucose, and increased hepatic ketogenesis in mice lacking fatty acid-binding protein 4 (FABP4) and FABP5 (DKO)...
September 20, 2017: Metabolism: Clinical and Experimental
https://www.readbyqxmd.com/read/28922393/a-set-of-nutrient-limitations-trigger-yeast-cell-death-in-a-nitrogen-dependent-manner-during-wine-alcoholic-fermentation
#17
Camille Duc, Martine Pradal, Isabelle Sanchez, Jessica Noble, Catherine Tesnière, Bruno Blondin
Yeast cell death can occur during wine alcoholic fermentation. It is generally considered to result from ethanol stress that impacts membrane integrity. This cell death mainly occurs when grape musts processing reduces lipid availability, resulting in weaker membrane resistance to ethanol. However the mechanisms underlying cell death in these conditions remain unclear. We examined cell death occurrence considering yeast cells ability to elicit an appropriate response to a given nutrient limitation and thus survive starvation...
2017: PloS One
https://www.readbyqxmd.com/read/28918449/immunochemical-study-of-the-effect-of-f2glc-on-glycogen-synthase-translocation-and-glycogen-synthesis-in-isolated-rat-hepatocytes
#18
J M Fernández-Novell, M Díaz-Lobo
The compound 2-deoxy-2-fluoro-α-D-glucopyranosyl fluoride (F2Glc), which is a nonmetabolized superior glucose analogue, is a potent inhibitor of glycogen phosphorylase and pharmacological properties are reported. Glycogen phosphorylase (GP) and glycogen synthase (GS) are responsible of the degradation and synthesis, respectively, of glycogen which is a polymer of glucose units that provides a readily available source of energy in mammals. GP and GS are two key enzymes that modulate cellular glucose and glycogen levels; therefore, these proteins are suggested as potential targets for the treatment of diseases related to glycogen metabolism disorders...
September 16, 2017: Applied Biochemistry and Biotechnology
https://www.readbyqxmd.com/read/28916327/glycogen-metabolism-in-brain-and-neurons-astrocytes-metabolic-cooperation-can-be-altered-by-pre-and-neonatal-lead-pb-exposure
#19
Irena Baranowska-Bosiacka, Anna Falkowska, Izabela Gutowska, Magdalena Gąssowska, Agnieszka Kolasa-Wołosiuk, Maciej Tarnowski, Karina Chibowska, Marta Goschorska, Anna Lubkowska, Dariusz Chlubek
Lead (Pb) is an environmental neurotoxin which particularly affects the developing brain but the molecular mechanism of its neurotoxicity still needs clarification. The aim of this paper was to examine whether pre- and neonatal exposure to Pb (concentration of Pb in rat offspring blood below the "threshold level") may affect the brain's energy metabolism in neurons and astrocytes via the amount of available glycogen. We investigated the glycogen concentration in the brain, as well as the expression of the key enzymes involved in glycogen metabolism in brain: glycogen synthase 1 (Gys1), glycogen phosphorylase (PYGM, an isoform active in astrocytes; and PYGB, an isoform active in neurons) and phosphorylase kinase β (PHKB)...
September 1, 2017: Toxicology
https://www.readbyqxmd.com/read/28905000/transcriptome-analysis-of-polyhydroxybutyrate-cycle-mutants-reveals-discrete-loci-connecting-nitrogen-utilization-and-carbon-storage-in-sinorhizobium-meliloti
#20
Maya D'Alessio, Ricardo Nordeste, Andrew C Doxey, Trevor C Charles
Polyhydroxybutyrate (PHB) and glycogen polymers are produced by bacteria as carbon storage compounds under unbalanced growth conditions. To gain insights into the transcriptional mechanisms controlling carbon storage in Sinorhizobium meliloti, we investigated the global transcriptomic response to the genetic disruption of key genes in PHB synthesis and degradation and in glycogen synthesis. Under both nitrogen-limited and balanced growth conditions, transcriptomic analysis was performed with genetic mutants deficient in PHB synthesis (phbA, phbB, phbAB, and phbC), PHB degradation (bdhA, phaZ, and acsA2), and glycogen synthesis (glgA1)...
September 2017: MSystems
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