65,213 results match your criteria Glycogen Storage Diseases Types I-VII


Label-free molecular mapping and assessment of glycogen in C. elegans.

Analyst 2019 Feb 22. Epub 2019 Feb 22.

Leibniz Institute of Photonic Technology Jena, Germany.

Caenorhabditis elegans is an animal model frequently used in research on the effects of metabolism on organismal aging. This comes with a requirement for methods to investigate metabolite content, turnover, and distribution. The aim of our study was to assess the use of a label-free approach to determine both content and distribution of glycogen, the storage form of glucose, in C. Read More

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http://dx.doi.org/10.1039/c8an02351dDOI Listing
February 2019

Liver cancer: WISP3 suppresses hepatocellular carcinoma progression by negative regulation of β-catenin/TCF/LEF signalling.

Cell Prolif 2019 Feb 22:e12583. Epub 2019 Feb 22.

CAS Key Laboratory of Nutrition, Metabolism and Food Safety, Shanghai Institute of Nutrition and Health, Shanghai Institutes for Biological Sciences, Chinese Academy of Sciences, Shanghai, China.

Objectives: Wnt1-inducible signalling pathway protein 3 (WISP3/CCN6) belongs to the CCN (CYR61/CTGF/NOV) family of proteins, dysregulation of this family contributed to the tumorigenicity of various tumours. In this study, we need to explore its role in hepatocellular carcinoma that remains largely elusive.

Materials And Methods: The expression of WISP3/CCN6 was analysed by qRT-PCR and Western blotting. Read More

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http://dx.doi.org/10.1111/cpr.12583DOI Listing
February 2019

Metformin promotes survivin degradation through AMPK/PKA/GSK-3β-axis in non-small cell lung cancer.

J Cell Biochem 2019 Feb 21. Epub 2019 Feb 21.

Department of Pulmonary and Critical Care Medicine, The First Affiliated Hospital of Kunming Medical University, Kunming, P.R. China.

Metformin, a first-line antidiabetic drug, has been reported with anticancer activities in many types of cancer. However, its molecular mechanisms remain largely unknown. As a member of inhibitor of apoptosis proteins, survivin plays an important role in the regulation of cell death. Read More

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http://dx.doi.org/10.1002/jcb.28470DOI Listing
February 2019

Late and Severe Myopathy in a Patient With Glycogenosis VII Worsened by Cyclosporine and Amiodarone.

Front Neurol 2019 7;10:77. Epub 2019 Feb 7.

Department of Clinical and Experimental Medicine, UOC di Neurologia e Malattie Neuromuscolari, University of Messina, Messina, Italy.

Glycogenosis VII (GSD VII) is a rare autosomal recessive glycogen storage disorder caused by mutations in the gene encoding the phosphofructokinase (PFK) enzyme. A classical form with exercise intolerance, contractures, and myoglobinuria, a severe multisystem infantile form, an hemolytic variant and a late-onset form usually presenting with muscle pain and mild fixed proximal weakness have been reported. We describe a 65-year-old man affected by muscle PFK deficiency who, since the age of 33, presented with exercise intolerance and myoglobinuria. Read More

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http://dx.doi.org/10.3389/fneur.2019.00077DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6374292PMC
February 2019

Fatal dysfunction and disintegration of thrombin-stimulated platelets.

Haematologica 2019 Feb 21. Epub 2019 Feb 21.

University of Pennsylvania Perelman School of Medicine, Philadelphia, USA

Platelets play a key role in formation of hemostatic clots and obstructive thrombi as well as in other biological processes. In response to physiological stimulants, including thrombin, platelets change their morphology, express adhesive molecules, undergo aggregation, and secrete bioactive substances, but their subsequent fate is largely unknown. Here we examine late-stage structural, metabolic, and functional consequences of thrombin-induced platelet activation. Read More

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http://dx.doi.org/10.3324/haematol.2018.202309DOI Listing
February 2019

Acute pancreatitis in a patient with glycogen storage disease type 1a.

Pediatr Neonatol 2019 Jan 29. Epub 2019 Jan 29.

Department of Pediatrics, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan.

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http://dx.doi.org/10.1016/j.pedneo.2019.01.009DOI Listing
January 2019

Low dose lithium supplementation activates Wnt/β-catenin signalling and increases bone OPG/RANKL ratio in mice.

Biochem Biophys Res Commun 2019 Feb 18. Epub 2019 Feb 18.

Department of Kinesiology, Brock University, St. Catharines, Ontario, Canada; Centre for Bone and Muscle Health, Brock University, St. Catharines, Ontario, Canada. Electronic address:

Lithium, a well-known inhibitor of glycogen synthase kinase-3β (GSK3β), can improve bone formation by activating the Wnt/β-catenin signalling pathway. However, most studies have used higher doses of lithium, which potentially have adverse effects. Herein, we report that low dose lithium supplementation (10 mg/kg/d for 6 weeks) in mice results in a serum lithium concentration of 0. Read More

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http://dx.doi.org/10.1016/j.bbrc.2019.02.066DOI Listing
February 2019

Glycogen Synthase Kinase 3 Beta Controls Presenilin-1-Mediated Endoplasmic Reticulum Ca²⁺ Leak Directed to Mitochondria in Pancreatic Islets and β-Cells.

Cell Physiol Biochem 2019 18;52(1):57-75. Epub 2019 Feb 18.

Molecular Biology and Biochemistry, Gottfried Schatz Research Center for Cellular Signaling, Metabolism & Aging, Medical University of Graz, Graz, Austria.

Background/aims: In pancreatic β-cells, the intracellular Ca²⁺ homeostasis is an essential regulator of the cells major functions. The endoplasmic reticulum (ER) as interactive intracellular Ca²⁺ store balances cellular Ca²⁺. In this study basal ER Ca²⁺ homeostasis was evaluated in order to reveal potential β-cell-specificity of ER Ca²⁺ handling and its consequences for mitochondrial Ca²⁺, ATP and respiration. Read More

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http://dx.doi.org/10.33594/000000005DOI Listing
February 2019

Enhanced myocardial protection in cardiac donation after circulatory death using Intralipid postconditioning in a porcine model.

Can J Anaesth 2019 Feb 21. Epub 2019 Feb 21.

Department of Anesthesiology and Pain Medicine, University of Alberta, Edmonton, AB, T6G 2G3, Canada.

Purpose: Intralipid (ILE), a clinically used lipid emulsion, reduces ischemia-reperfusion (IR) injury in healthy and infarct-remodelled rat hearts. We tested whether ILE is also cardioprotective in large porcine hearts in the context of the donation after circulatory death (DCD) model, where human hearts are procured for transplantation after cardiac arrest and thus are exposed to significant IR injury.

Methods: After induction of anesthesia, surgical preparation, termination of ventilator support, and cardiac arrest, hearts of female pigs were procured following a 15 min standoff period, with an optimized normokalemic crystalloid adenosine-lidocaine cardioplegia. Read More

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http://dx.doi.org/10.1007/s12630-019-01322-xDOI Listing
February 2019

Crocin-protected malathion-induced spatial memory deficits by inhibiting TAU protein hyperphosphorylation and antiapoptotic effects.

Nutr Neurosci 2019 Feb 21:1-16. Epub 2019 Feb 21.

a Department of Pharmacodynamics and Toxicology, School of Pharmacy , Mashhad University of Medical Sciences , Mashhad , Iran.

Organophosphorus compounds are widely used in agriculture. Epidemiological studies propose that pesticide exposure is a risk factor for Alzheimer's disease (AD), but the mechanisms are unclear. Here, we investigated the impact of malathion exposure on the cognitive ability and the underlying mechanisms in rats. Read More

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http://dx.doi.org/10.1080/1028415X.2018.1492772DOI Listing
February 2019

A 1:1 exercise-to-rest period ratio needed by animals to restore energy sources and replenish anti-oxidative status after exercise.

Nutr Res Pract 2019 Feb 6;13(1):17-22. Epub 2018 Dec 6.

Department of Food and Nutrition, Duksung Women's University, 33 Samyangro 144, Dobong-Gu, Seoul, 01369, South Korea.

Background/objectives: Successful recovery of an animal from exercise is essential, especially prior to the next exercise session. This study was conducted to find an effective exercise-to-rest period ratio for the restoration of energy sources and replenishment of anti-oxidative status in tissue after exercise.

Materials/methods: Thirty-two rats were assigned to either non-training or training exercise groups for 5 weeks. Read More

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http://dx.doi.org/10.4162/nrp.2019.13.1.17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6369116PMC
February 2019

Distinct Functions for Mammalian CLASP1 and -2 During Neurite and Axon Elongation.

Front Cell Neurosci 2019 29;13. Epub 2019 Jan 29.

Department of Cell Biology, Erasmus Medical Center, University Medical Center Rotterdam, Rotterdam, Netherlands.

Mammalian cytoplasmic linker associated protein 1 and -2 (CLASP1 and -2) are microtubule (MT) plus-end tracking proteins that selectively stabilize MTs at the edge of cells and that promote MT nucleation and growth at the Golgi, thereby sustaining cell polarity. analysis has shown that CLASPs are MT growth promoting factors. To date, a single CLASP1 isoform (called CLASP1α) has been described, whereas three CLASP2 isoforms are known (CLASP2α, -β, and -γ). Read More

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http://dx.doi.org/10.3389/fncel.2019.00005DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6373834PMC
January 2019

Neuroprotective potential of ketamine prevents developing brain structure impairment and alteration of neurocognitive function induced via isoflurane through the PI3K/AKT/GSK-3β pathway.

Drug Des Devel Ther 2019 4;13:501-512. Epub 2019 Feb 4.

Department of Anesthesiology, Shandong Provincial Hospital Affiliated to Shandong University, Jinan, Shandong Province 250021, People's Republic of China,

Background: The aim of the current experimental study was to scrutinize the neuroprotective effect of ketamine on the isoflurane (iso)-induced cognitive dysfunction in rats via phosphoinositide 3 kinase (PI3K)/protein kinase B (AKT)/glycogen synthase kinase 3β (GSK-3β) pathway.

Materials And Methods: Sprague-Dawley rats were used for the current experimental study. The rats were divided into six groups and rats were treated with ketamine and memantine. Read More

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http://dx.doi.org/10.2147/DDDT.S188636DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6366353PMC
February 2019

The impact of Pompe disease on smooth muscle: a review.

J Smooth Muscle Res 2018 ;54(0):100-118

Department of Pediatrics, School of Medicine, Duke University, Durham, NC, USA.

Pompe disease (OMIM 232300) is an autosomal recessive disorder caused by mutations in the gene encoding acid α-glucosidase (GAA) (EC 3.2.1. Read More

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http://dx.doi.org/10.1540/jsmr.54.100DOI Listing
January 2018

[Steroid-induced diabetes in the paediatric population].

Pediatr Endocrinol Diabetes Metab 2018 ;2018(3):136-139

Steroid-induced diabetes is a rare disease in the paediatric population. High doses of corticosteroids are used in diseases such as acute lymphoblastic leukaemia (ALL), lymphomas, or connective tissue diseases. Post-steroid hyperglycaemia arises as a result of increased gluconeogenesis and increased glycogen synthesis. Read More

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http://dx.doi.org/10.5114/pedm.2018.80995DOI Listing
January 2018

Evaluation of biochemical mechanisms of anti-diabetic functions of Anisomeles malabarica.

Biomed Pharmacother 2019 Feb 19;112:108598. Epub 2019 Feb 19.

Department of Biochemistry, Sri Venkateswara University, Tirupati, AP, India. Electronic address:

Anisomeles malabarica (AM) is an aromatic plant traditionally used for the treatment of diabetes mellitus in India. Following bioassay guided fractionation, we recently identified an active fraction of AM (AMAF, with potential mix of active principles) that showed significant antihyperglycemic and antihyperlipidemic activities. In addition, AMAF treatment improved insulin levels. Read More

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http://dx.doi.org/10.1016/j.biopha.2019.01.059DOI Listing
February 2019

Changes in biochemical contents and survival rates of two stored product moths under different thermal regimes.

J Therm Biol 2019 Feb 25;80:7-15. Epub 2018 Dec 25.

Pistachio Safety Research Center, Rafsanjan University of Medical Sciences, Rafsanjan, Iran.

Plodia interpunctella (Hübner) and Ectomyelois ceratoniae (Zeller) (Lepidoptera: Pyralidae) are two destructive pests of stored products. Survival and physiological changes of last instar larvae were investigated under different thermal regimes [i.e. Read More

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http://dx.doi.org/10.1016/j.jtherbio.2018.12.022DOI Listing
February 2019

PI3K signaling: a crossroads of metabolic regulation.

Expert Rev Endocrinol Metab 2009 Jul;4(4):349-357

e Professor, Molecular Biotechnology Center, Via Nizza 52, 10126 Turin, Italy.

Insulin exerts a fundamental role in glucose metabolism. Several lines of evidence have established PI3Ks as crucial signaling crossroads of metabolic regulation. These kinases play a key role in glucose homeostasis through the generation of lipid secondary messengers upon membrane receptor activation, thus regulating liver gluconeogenesis and glycogen synthesis. Read More

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http://dx.doi.org/10.1586/eem.09.19DOI Listing

Vaccarin ameliorates insulin resistance and steatosis by activating the AMPK signaling pathway.

Eur J Pharmacol 2019 Feb 16. Epub 2019 Feb 16.

Department of Pharmacology, Yong Loo Lin School of Medicine, National University of Singapore, Singapore, 117597, Singapore. Electronic address:

Destructive glucose and lipid metabolism in the liver is a crucial characteristic of type 2 diabetes mellitus (T2DM), eventually leading to insulin resistance and subsequent hyperglycemia and hyperlipidemia. Vaccarin is an active flavonoid glycoside associated with various biological functions, but its effects on glucose and lipid metabolic disorder in T2DM are still unclear. In this study, glucosamine (GlcN) and free fatty acids (FFAs, oleate/palmitate, 2:1 ratio) were applied to mimic insulin resistance and lipid deposition in HepG2 cells, respectively. Read More

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http://dx.doi.org/10.1016/j.ejphar.2019.02.029DOI Listing
February 2019

Loss of growth hormone-mediated signal transducer and activator of transcription 5 (STAT5) signaling in mice results in insulin sensitivity with obesity.

FASEB J 2019 Feb 19:fj201802328R. Epub 2019 Feb 19.

Institute for Molecular Bioscience, The University of Queensland, Brisbane, Queensland, Australia.

Growth hormone (GH) has an important function as an insulin antagonist with elevated insulin sensitivity evident in humans and mice lacking a functional GH receptor (GHR). We sought the molecular basis for this sensitivity by utilizing a panel of mice possessing specific deletions of GHR signaling pathways. Metabolic clamps and glucose homeostasis tests were undertaken in these obese adult C57BL/6 male mice, which indicated impaired hepatic gluconeogenesis. Read More

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http://dx.doi.org/10.1096/fj.201802328RDOI Listing
February 2019

Histone acetyltransferase MoHat1 acetylates autophagy-related proteins MoAtg3 and MoAtg9 to orchestrate functional appressorium formation and pathogenicity in Magnaporthe oryzae.

Autophagy 2019 Feb 18:1-24. Epub 2019 Feb 18.

a Department of Plant Pathology, College of Plant Protection , Nanjing Agricultural University , Nanjing , China.

Macroautophagy/autophagy is critical for normal appressorium formation and pathogenicity of the rice blast fungus Magnaporthe oryzae, but the molecular base of autophagy linked to pathogenicity remains elusive in this or other pathogenic fungi. We found that MoHat1, a histone acetyltransferase (HAT) homolog, had a role in the regulation of autophagy through the acetylation of autophagy related proteins MoAtg3 and MoAtg9. We also found that MoHat1 was subject to regulation by the protein kinase MoGsk1 that modulated the translocation of MoHat1 from the nucleus to the cytoplasm with the assistance of MoSsb1, a protein chaperone. Read More

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http://dx.doi.org/10.1080/15548627.2019.1580104DOI Listing
February 2019

Impacts of S-metolachlor and terbuthylazine in fatty acid and carbohydrate composition of the benthic clam Scrobicularia plana.

Ecotoxicol Environ Saf 2019 Feb 15;173:293-304. Epub 2019 Feb 15.

MARE - Marine and Environmental Sciences Centre, Department of Life Sciences, Faculty of Sciences and Technology, University of Coimbra, 3004-517 Coimbra, Portugal; Department of Biology & CESAM, University of Aveiro, 3810-193 Aveiro, Portugal.

S-metolachlor (SMOC) and terbuthylazine (TBA) are herbicides that commonly appear as active ingredients (a.i.) in the composition of plant protection products. Read More

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http://dx.doi.org/10.1016/j.ecoenv.2019.02.034DOI Listing
February 2019

Glycogen synthase kinase-3 inhibitor as a multi-targeting anti-rheumatoid drug.

Biochem Pharmacol 2019 Feb 15. Epub 2019 Feb 15.

Department of Pharmacology, School of Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan. Electronic address:

Rheumatoid arthritis (RA) is a chronic inflammatory joint disease that causes swelling, bone erosion, and joint disorder. Patients with RA therefore suffer from pain and physiological disability, and have a decreased quality of life. During the progression of RA, many different types of cells and inflammatory factors influence each other with an important role. Read More

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http://dx.doi.org/10.1016/j.bcp.2019.02.020DOI Listing
February 2019

A Potential Role for α-Amylase in Amyloid-β-Induced Astrocytic Glycogenolysis and Activation.

J Alzheimers Dis 2019 Feb 11. Epub 2019 Feb 11.

Department of Clinical Sciences Malmö, Clinical Memory Research Unit, Lund University, Malmö, Sweden.

Background: Astrocytes produce and store the energy reserve glycogen. However, abnormal large glycogen units accumulate if the production or degradation of glycogen is disturbed, a finding often seen in patients with Alzheimer's disease (AD). We have shown increased activity of glycogen degrading α-amylase in AD patients and α-amylase positive glial cells adjacent to AD characteristic amyloid-β (Aβ) plaques. Read More

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http://dx.doi.org/10.3233/JAD-180997DOI Listing
February 2019

Adverse impact of carbon tetrachloride on metabolic function in mice.

J Cell Biochem 2019 Feb 18. Epub 2019 Feb 18.

College of Pharmacy, Guangxi Medical University, Guangxi, Nanning, P. R. China.

Carbon tetrachloride (CCl ), a potent hepatotoxin, is linked to the histopathological outcomes of inflammatory or oxidative stress, and cell death. However, further study of additional dysmetabolism induced by CCl toxicant has not yet been investigated. In current study, chronical and acute exposures of CCl in mice were used to unmask the biological molecular mechanism responsible for insulin-dependent metabolic disorder. Read More

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http://dx.doi.org/10.1002/jcb.28481DOI Listing
February 2019

Pathway-oriented action of dietary essential oils to prevent muscle protein oxidation and texture deterioration of farmed rainbow trout.

Animal 2019 Feb 18:1-12. Epub 2019 Feb 18.

2Instituto de Investigaciones Marinas,Consejo Superior de Investigaciones Científicas (IIM-CSIC),Eduardo Cabello 6,E-36208 Vigo,Spain.

Recently, great attention has been directed towards the use of essential oils from aromatic plants as antimicrobials and antioxidant in food matrix. Fish is well known to be a high perishable food. Indeed, fish muscle is susceptible to suffer protein and lipid oxidation during frozen storage, which can lead to the development of softening and undesirable volatile molecules. Read More

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http://dx.doi.org/10.1017/S1751731119000016DOI Listing
February 2019

Ultrastructural Study of Clitoral Cavernous Tissue and Clitoral Blood Flow From Type 1 Diabetic Premenopausal Women on Phosphodiesterase-5 Inhibitor.

J Sex Med 2019 Feb 14. Epub 2019 Feb 14.

Unit of Ultrastructural Pathology, Department of Human Pathology, University of Messina, Messina, Italy.

Background: The effects of phosphodiesterase-type 5 (PDE5) inhibitors on the in vivo clitoral structure of women with diabetes have never been investigated.

Aim: To study the in vivo structural and hemodynamic changes of the clitoris in premenopausal women with type 1 diabetes on PDE5 inhibitors.

Methods: 38 premenopausal women with type 1 diabetes aged 36 -46 years. Read More

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http://dx.doi.org/10.1016/j.jsxm.2019.01.003DOI Listing
February 2019

Carbohydrate catabolic capability of a Flavobacteriia bacterium isolated from hadal water.

Syst Appl Microbiol 2019 Jan 29. Epub 2019 Jan 29.

MOE Key Laboratory of Marine Genetics and Breeding, College of Marine Life Sciences, Ocean University of China, 5 Yushan Road, Qingdao 266003, China; Laboratory for Marine Ecology and Environmental Science, Qingdao National Laboratory for Marine Science and Technology, Qingdao 266071, China. Electronic address:

Flavobacteriia are abundant in many marine environments including hadal waters, as demonstrated recently. However, it is unclear how this flavobacterial population adapts to hadal conditions. In this study, extensive comparative genomic analyses were performed for the flavobacterial strain Euzebyella marina RN62 isolated from the Mariana Trench hadal water in low abundance. Read More

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http://dx.doi.org/10.1016/j.syapm.2019.01.002DOI Listing
January 2019

High level of dietary soybean oil affects the glucose and lipid metabolism in large yellow croaker Larimichthys crocea through the insulin-mediated PI3K/AKT signaling pathway.

Comp Biochem Physiol B Biochem Mol Biol 2019 Feb 14. Epub 2019 Feb 14.

The Key Laboratory of Mariculture (Ministry of Education), The Key Laboratory of Aquaculture Nutrition and Feeds (Ministry of Agriculture), Fisheries College, Ocean University of China, Qingdao 266003, China; Laboratory for Marine Fisheries Science and Food Production Processes, Qingdao National Laboratory for Marine Science and Technology, Wen Hai Road, Qingdao 266237, China.

The present study was conducted to investigate the metabolic responses of glucose and lipid in large yellow croaker Larimichthys crocea (initial weight, 36.80 ± 0.39 g) to high level of dietary soybean oil. Read More

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http://dx.doi.org/10.1016/j.cbpb.2018.12.003DOI Listing
February 2019

Chemical genetic screen identifies Gapex-5/GAPVD1 and STBD1 as novel AMPK substrates.

Cell Signal 2019 Feb 14. Epub 2019 Feb 14.

Nestlé Institute of Health Sciences SA, Ecole Polytechnique Fédérale de Lausanne (EPFL), Innovation Park, bâtiment G, 1015 Lausanne, Switzerland; School of Life Sciences, EPFL, 1015 Lausanne, Switzerland. Electronic address:

AMP-activated protein kinase (AMPK) is a key regulator of cellular energy homeostasis, acting as a sensor of energy and nutrient status. As such, AMPK is considered a promising drug target for treatment of medical conditions particularly associated with metabolic dysfunctions. To better understand the downstream effectors and physiological consequences of AMPK activation, we have employed a chemical genetic screen in mouse primary hepatocytes in an attempt to identify novel AMPK targets. Read More

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http://dx.doi.org/10.1016/j.cellsig.2019.02.001DOI Listing
February 2019

Control and regulation of the pyrophosphate-dependent glucose metabolism in Entamoeba histolytica.

Mol Biochem Parasitol 2019 Feb 14. Epub 2019 Feb 14.

Departamento de Bioquímica, Instituto Nacional de Cardiología Ignacio Chávez, Ciudad de México, 14080, Mexico.

Entamoeba histolytica has neither Krebs cycle nor oxidative phosphorylation activities; therefore, glycolysis is the main pathway for ATP supply and provision of carbon skeleton precursors for the synthesis of macromolecules. Glucose is metabolized through fermentative glycolysis, producing ethanol as its main end-product as well as some acetate. Amoebal glycolysis markedly differs from the typical Embden-Meyerhof-Parnas pathway present in human cells: (i) by the use of inorganic pyrophosphate, instead of ATP, as the high-energy phospho group donor; (ii) with one exception, the pathway enzymes can catalyze reversible reactions under physiological conditions; (iii) there is no allosteric regulation and sigmoidal kinetic behavior of key enzymes; and (iv) the presence of some glycolytic and fermentation enzymes similar to those of anaerobic bacteria. Read More

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http://dx.doi.org/10.1016/j.molbiopara.2019.02.002DOI Listing
February 2019

The impacts of warming on the toxicity of carbon nanotubes in mussels.

Mar Environ Res 2019 Jan 29. Epub 2019 Jan 29.

Department of Biology & Center for Environmental and Marine Studies (CESAM), University of Aveiro, 3810-193, Aveiro, Portugal. Electronic address:

With the increased production and research on nanoparticles, the presence of carbon nanotubes (CNTs) in aquatic systems is very likely to increase. Although it has been shown that CNTs may cause toxicity in marine organisms, to our knowledge, the possible impacts under global temperature increase is still unknown. For this reason, biochemical and physiological impacts induced in Mytilus galloprovincialis due to the presence of functionalized multi-walled CNTs (f-MWCNTs) and increased temperature were investigated in the present study. Read More

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http://dx.doi.org/10.1016/j.marenvres.2019.01.013DOI Listing
January 2019

The influence of temperature on the effects induced by Triclosan and Diclofenac in mussels.

Sci Total Environ 2019 Jan 16;663:992-999. Epub 2019 Jan 16.

Instituto de Ciencias del Mar ICM-CSIC, E-08003 Barcelona, Spain.

Little is known about the consequences of exposure to pharmaceuticals and personal care products (PPCPs) in elevated temperatures associated with climate change. To increase the knowledge on this topic, Mytilus galloprovincialis mussels were exposed to 1.0μg/L of either the antimicrobial Triclosan (TCS) or the anti-inflammatory drug Diclofenac (DIC), at control (17°C) and 4°C raised (21°C) temperatures for 28days. Read More

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http://dx.doi.org/10.1016/j.scitotenv.2019.01.189DOI Listing
January 2019
2 Reads

VEGF-C attenuates ischemia reperfusion injury of liver graft in rats.

Transpl Immunol 2019 Feb 13. Epub 2019 Feb 13.

Chongqing Key Laboratory of Hepatobiliary Surgery, The Second Affiliated Hospital of Chongqing Medical University, Chongqing, China; Department of Hepatobiliary Surgery, The Second Affiliated Hospital of Chongqing Medical University, Chongqing, China. Electronic address:

Background: Vascular endothelial growth factor receptor-3 (VEGFR-3) / vascular endothelial growth factor -c (VEGF-C) signaling is reported to negatively regulate TLR4-triggered inflammation of macrophages. This study aims to clarify whether the VEGFR-3/VEGF-C signaling can suppress Kupffer cells (KCs) activation and attenuate hepatic ischemia-reperfusion injury (IRI) after liver transplantation.

Methods: A rat model of liver transplantation was performed. Read More

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http://dx.doi.org/10.1016/j.trim.2019.02.004DOI Listing
February 2019
2 Reads

Complement C3a receptor antagonist attenuates tau hyperphosphorylation via glycogen synthase kinase 3β signaling pathways.

Eur J Pharmacol 2019 Feb 13. Epub 2019 Feb 13.

Department of Neurology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, China. Electronic address:

Neurofibrillary tangles aggregated from hyperphosphorylated tau protein are the main pathological feature of Alzheimer's disease (AD). Complement C3 (or C3a) is the core component of the complement system and is associated with AD pathological processes. However, it remains unclear whether C3a or the C3a receptor has any effect on tau phosphorylation. Read More

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http://dx.doi.org/10.1016/j.ejphar.2019.02.020DOI Listing
February 2019

miR-126 regulates glycogen trophoblast proliferation and DNA methylation in the murine placenta.

Dev Biol 2019 Feb 14. Epub 2019 Feb 14.

Department of Cell and Developmental Biology, Weill Cornell Medical College, 1300 York Avenue, Box 60, New York, NY 10065, United States. Electronic address:

A functional placenta develops through a delicate interplay of its vascular and trophoblast compartments. We have identified a previously unknown expression domain for the endothelial-specific microRNA miR-126 in trophoblasts of murine and human placentas. Here, we determine the role of miR-126 in placental development using a mouse model with a targeted deletion of miR-126. Read More

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http://dx.doi.org/10.1016/j.ydbio.2019.01.019DOI Listing
February 2019
1 Read

Safety, tolerability, pharmacokinetics, pharmacodynamics, and exploratory efficacy of the novel enzyme replacement therapy avalglucosidase alfa (neoGAA) in treatment-naïve and alglucosidase alfa-treated patients with late-onset Pompe disease: A phase 1, open-label, multicenter, multinational, ascending dose study.

Neuromuscul Disord 2018 Dec 17. Epub 2018 Dec 17.

Erasmus Medical Center, Pompe Center, Rotterdam, The Netherlands.

This multicenter/multinational, open-label, ascending-dose study (NCT01898364) evaluated safety, tolerability, pharmacokinetics, pharmacodynamics, and exploratory efficacy of repeat-dose avalglucosidase alfa (neoGAA), a second-generation, recombinant acid α-glucosidase replacement therapy, in late-onset Pompe disease (LOPD). Patients ≥18 years, alglucosidase alfa naïve (Naïve) or previously receiving alglucosidase alfa for ≥9 months (Switch), with baseline FVC ≥50% predicted and independently ambulatory, received every-other-week avalglucosidase alfa 5, 10, or 20 mg/kg over 24 weeks. 9/10 Naïve and 12/14 Switch patients completed the study. Read More

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http://dx.doi.org/10.1016/j.nmd.2018.12.004DOI Listing
December 2018
1 Read

Reevaluating the pathogenicity of the mutation c.1194 +5 G>A in GAA gene by functional analysis of RNA in a 61-year-old woman diagnosed with Pompe disease by muscle biopsy.

Neuromuscul Disord 2018 Dec 15. Epub 2018 Dec 15.

Unidad de Oncogenética Molecular, Instituto de Genética Médica y Molecular (INGEMM), Edificio Quirúrgico Planta-2, Hospital Universitario La Paz, 28046 Madrid, Spain; Department of Biochemistry, Faculty of Medicine, Autonoma University of Madrid, 28046 Madrid, Spain. Electronic address:

Glycogen storage disease type II, or Pompe disease, is an autosomal recessive disorder caused by deficiency of lysosomal acid alpha-glucosidase (GAA). We performed genetic analysis to confirm the diagnosis of Pompe disease in a 61-year-old patient with progressive weakness in extremities, severe Sleep Apnea-Hypopnea Syndrome, a significant reduction of alpha-glucosidase in liquid sample of peripheral blood and muscular biopsy diagnosis. GAA gene sequencing showed the patient is homozygous for the splice-site mutation c. Read More

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http://dx.doi.org/10.1016/j.nmd.2018.12.003DOI Listing
December 2018

Control of invasive sea lampreys using the piscicides TFM and niclosamide: Toxicology, successes & future prospects.

Aquat Toxicol 2018 Dec 21. Epub 2018 Dec 21.

Department of Biology & Laurier Institute for Water Science, Wilfrid Laurier University, Waterloo, Ontario, N2L 3C5, Canada.

The invasion of the Laurentian Great Lakes of North America by sea lampreys (Petromyzon marinus) in the early 20th century contributed to the depletion of commercial, recreational and culturally important fish populations, devastating the economies of communities that relied on the fishery. Sea lamprey populations were subsequently controlled using an aggressive integrated pest-management program which employed barriers and traps to prevent sea lamprey from migrating to their spawning grounds and the use of the piscicides (lampricides) 3-trifluoromethyl-4-nitrophenol (TFM) and niclosamide to eliminate larval sea lampreys from their nursery streams. Although sea lampreys have not been eradicated from the Great Lakes, populations have been suppressed to less than 10% of their peak numbers in the mid-1900s. Read More

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http://dx.doi.org/10.1016/j.aquatox.2018.12.012DOI Listing
December 2018
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Clinical Features, Survival and Prognostic Factors of Glycogen-Rich Clear Cell Carcinoma (GRCC) of the Breast in the U.S. Population.

J Clin Med 2019 Feb 14;8(2). Epub 2019 Feb 14.

Department of Molecular and Cellular Biochemistry, University of Kentucky College of Medicine, Lexington, KY 40536, USA.

The World Health Organization (WHO) defines glycogen-rich clear cell carcinoma (GRCC) of the breast as a carcinoma with glycogen accumulation in more than 90% of its tumor cells. Due to the rarity of this disease, its reported survival and clinical associations have been inconsistent due to reliance on case reports and limited case series. As a result, the prognostic implication of this cancer subtype remains unclear. Read More

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http://dx.doi.org/10.3390/jcm8020246DOI Listing
February 2019
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Haralick texture feature analysis for quantifying radiation response heterogeneity in murine models observed using Raman spectroscopic mapping.

PLoS One 2019 15;14(2):e0212225. Epub 2019 Feb 15.

The Department of Physics, University of British Columbia Okanagan Campus, Kelowna, BC, Canada.

Tumour heterogeneity plays a large role in the response of tumour tissues to radiation therapy. Inherent biological, physical, and even dose deposition heterogeneity all play a role in the resultant observed response. We here implement the use of Haralick textural analysis to quantify the observed glycogen production response, as observed via Raman spectroscopic mapping, of tumours irradiated within a murine model. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0212225PLOS
February 2019

Denitrification activity of polyphosphate accumulating organisms (PAOs) in full-scale wastewater treatment plants.

Water Sci Technol 2018 Dec;78(12):2449-2458

Chemistry Department FCT-UNL, UCIBIO, REQUIMTE, 2829-516 Caparica, Portugal.

A comprehensive assessment of full-scale enhanced biological phosphorus removal (EBPR) plants (five plants, 19 independent tests) was undertaken to determine their effectiveness in terms of aerobic and anoxic P removal. By comparing parallel P uptake tests under only aerobic or under anoxic-aerobic conditions, results revealed that introducing an anoxic stage led to an overall P removal of on average 90% of the P removed under only aerobic conditions. This was achieved with negligible higher PHA and glycogen requirements, 30% lower overall oxygen consumption and with the simultaneous removal of nitrate, reducing up to an estimate of 70% of carbon requirements for simultaneous N and P removal. Read More

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http://dx.doi.org/10.2166/wst.2018.517DOI Listing
December 2018

FcαRI Dynamics Are Regulated by GSK-3 and PKCζ During Cytokine Mediated Inside-Out Signaling.

Front Immunol 2018 31;9:3191. Epub 2019 Jan 31.

Laboratory of Translational Immunology, University Medical Center, Utrecht, Netherlands.

IgA binding to FcαRI (CD89) is rapidly enhanced by cytokine induced inside-out signaling. Dephosphorylation of serine 263 in the intracellular tail of FcαRI by PP2A and PI3K activation are instrumental in this process. To further investigate these signaling pathways, we targeted downstream kinases of PI3K. Read More

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http://dx.doi.org/10.3389/fimmu.2018.03191DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6365424PMC
January 2019

Antisense Inhibition of Glucagon Receptor by IONIS-GCGR Improves Type 2 Diabetes Without Increase in Hepatic Glycogen Content in Patients With Type 2 Diabetes on Stable Metformin Therapy.

Diabetes Care 2019 Feb 14. Epub 2019 Feb 14.

Ionis Pharmaceuticals, Inc., Carlsbad, CA

Objective: To evaluate the safety and efficacy of IONIS-GCGR, a 2'--methoxyethyl antisense oligonucleotide targeting the glucagon receptor (GCGR), and the underlying mechanism of liver transaminase increases in patients with type 2 diabetes on stable metformin therapy.

Research Design And Methods: In three phase 2, randomized, double-blind studies, patients with type 2 diabetes on metformin received weekly subcutaneous injections of IONIS-GCGR (50-200 mg) or placebo for 13 or 26 weeks.

Results: Significant reductions in HbA were observed after IONIS-GCGR treatment versus placebo at week 14 (-2. Read More

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http://dx.doi.org/10.2337/dc18-1343DOI Listing
February 2019

Distinct molecular signature of phospholamban p.Arg14del arrhythmogenic cardiomyopathy.

Cardiovasc Pathol 2018 Dec 21;40:2-6. Epub 2018 Dec 21.

University of Groningen, University Medical Center Groningen, Department of Pathology, Groningen, The Netherlands.

Phospholamban (PLN) p.Arg14del cardiomyopathy is characterized by a distinct arrhythmogenic biventricular phenotype that can be predominantly left ventricular, right ventricular, or both. Our aim was to further elucidate distinct features of this cardiomyopathy with respect to the distribution of desmosomal proteins observed by immunofluorescence (IF) in comparison to desmosomal arrhythmogenic cardiomyopathy and co-existent genetic variants. Read More

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http://dx.doi.org/10.1016/j.carpath.2018.12.006DOI Listing
December 2018
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Homoharringtonine potentiates the antileukemic activity of arsenic trioxide against acute myeloid leukemia cells.

Exp Cell Res 2019 Feb 11. Epub 2019 Feb 11.

Central Laboratory, The Union Hospital of Fujian Medical University, Fuzhou, China. Electronic address:

Relapse of minimal residual disease (MRD) is a major problem after conventional chemotherapy in patients with acute myeloid leukemia (AML). The bone marrow stroma can protect AML cells from insults of chemotherapy, partly contributing to AML relapse. Arsenic trioxide (ATO) is the main component of arsenical traditional Chinese medicines and has been widely used for the treatment of hematologic malignancies particularly acute promyelocytic leukemia over the past three decades. Read More

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http://dx.doi.org/10.1016/j.yexcr.2019.02.008DOI Listing
February 2019
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Insulin-like growth factor-I promotes hepatic differentiation of human adipose tissue-derived stem cells.

Cell Biol Int 2019 Feb 13. Epub 2019 Feb 13.

Department of Clinical Biochemistry, Faculty of Medicine, Hormozgan University of Medical Sciences, Bandar Abbas, Iran.

There are numerous studies which provide support for the use of human adipose tissue-derived stem cells (hASCs) to generate hepatocyte-like cells. However, the produced cells exhibit only a certain level of differentiation, mainly due to inefficient induction conditions. Therefore, based on the important role of insulin-like growth factor (IGF-I) in hepatic function and development, in the current study we evaluated the differentiation efficacy of the mentioned factor to induce hASCs into functional hepatocyte-like cells. Read More

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http://dx.doi.org/10.1002/cbin.11113DOI Listing
February 2019

Highly-expressed P2X7 receptor promotes growth and metastasis of human HOS/MNNG osteosarcoma cells via PI3K/Akt/GSK3β/β-catenin and mTOR/HIF1α/VEGF signaling.

Int J Cancer 2019 Feb 13. Epub 2019 Feb 13.

Department of Orthopedics, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Jiefang Avenue 1095, Wuhan 430030, China.

The P2X7 receptor, an ATP-gated ion channel, is critical for cancer cell growth, invasiveness, and angiogenesis. Previous studies indicate that P2X7 regulates osteoblast proliferation and osteodeposition and that high P2X7 expression has a pro-growth effect in osteosarcoma. However, how it functions in osteosarcoma cell growth and metastasis is not clear. Read More

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http://dx.doi.org/10.1002/ijc.32207DOI Listing
February 2019
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Interference of heavy metals present in the water of the Lagoa Tramandaí/RS on the carbohydrate metabolism of the GURI Sea Catfish (G. genidens) and Bay whiff (C. spilopterus).

Comp Biochem Physiol C Toxicol Pharmacol 2019 Feb 10;219:42-49. Epub 2019 Feb 10.

Unilasalle-Universidade Lassalle, Mestrado de Avaliação em Impactos Ambientais, Av. Victor Barreto, 2288, CEP 92010-000 Canoas, RS, Brazil.

This study aims to evaluate the contamination of the water of Lagoa Tramandaí on Genidens genidens and Citharichthys spilopterus residents of Lagoa Tramandaí, a region susceptible to environmental damage due to the release of domestic sewage from the municipalities bathed by the lagoon. Thereby, the biota suffers the interference of heavy metals, affecting their behavior and the metabolism of these individuals. The hepatic and muscular glycogen, glucose and plasma lactate of these species were analyzed, as well as the presence of heavy metals in lagoon water. Read More

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http://dx.doi.org/10.1016/j.cbpc.2019.01.007DOI Listing
February 2019

5'-Iodotubercidin represses expression, decreases cAMP levels, and suppresses human neuroblastoma cell growth.

J Biol Chem 2019 Feb 12. Epub 2019 Feb 12.

Departments of Pediatrics and Genetics, LSU Health Sciences Center, United States.

Insulinoma associated-1 (INSM1) is a key protein functioning as a transcriptional repressor in neuroendocrine differentiation and is activated by N-Myc in human neuroblastoma (NB). INSM1 modulates the phosphoinositide 3-kinase (PI3K)-AKT Ser/Thr kinase (AKT)-glycogen synthase kinase 3β (GSK3β) signaling pathway through a positive-feedback loop, resulting in N-Myc stabilization. Accordingly, INSM1 has emerged as a critical player closely associated with N-Myc in facilitating NB cell growth. Read More

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http://dx.doi.org/10.1074/jbc.RA118.006761DOI Listing
February 2019
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