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Glycogen storage

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https://www.readbyqxmd.com/read/29345438/evaluation-of-hepatic-glycogen-content-some-haematological-and-biochemical-parameters-of-alloxan-induced-diabetic-rats-treated-with-combinations-of-glibenclamide-and-g-latifolium-extract
#1
Patrick E Aba
Background Diabetes is associated with both biochemical and haematological complications. Combination therapy has been advocated to mitigate some of these complications. Aim This study was designed to investigate the effects of glibenclamide and Gongronema latifolium (GL) on hepatic glycogen content and haemato-biochemical parameters. Methods Thirty male Wistar rats were assigned into five groups of six rats each. Groups 2-5 rats received intraperitoneally, 160 mg/kg of alloxan monohydrate while group 1 rats served as normal control...
May 4, 2017: Journal of Complementary & Integrative Medicine
https://www.readbyqxmd.com/read/29330356/metabolic-and-molecular-changes-associated-with-the-increased-skeletal-muscle-insulin-action-24-48%C3%A2-h-after-exercise-in-young-and-old-humans
#2
REVIEW
Francis B Stephens, Kostas Tsintzas
The molecular and metabolic mechanisms underlying the increase in insulin sensitivity (i.e. increased insulin-stimulated skeletal muscle glucose uptake, phosphorylation and storage as glycogen) observed from 12 to 48 h following a single bout of exercise in humans remain unresolved. Moreover, whether these mechanisms differ with age is unclear. It is well established that a single bout of exercise increases the translocation of the glucose transporter, GLUT4, to the plasma membrane. Previous research using unilateral limb muscle contraction models in combination with hyperinsulinaemia has demonstrated that the increase in insulin sensitivity and glycogen synthesis 24 h after exercise is also associated with an increase in hexokinase II (HKII) mRNA and protein content, suggesting an increase in the capacity of the muscle to phosphorylate glucose and divert it towards glycogen synthesis...
January 12, 2018: Biochemical Society Transactions
https://www.readbyqxmd.com/read/29326002/prevalence-of-adult-pompe-disease-in-patients-with-proximal-myopathic-syndrome-and-undiagnosed-muscle-biopsy
#3
Amir Golsari, Arzoo Nasimzadah, Götz Thomalla, Sarah Keller, Christian Gerloff, Tim Magnus
We examined patients with limb-girdle muscle weakness and/or hyper-CKaemia and undiagnosed muscle biopsy for late onset Pompe disease (LOPD). Patients with an inconclusive limb-girdle muscle weakness who presented at our neuromuscular centre between 2005 and 2015 with undiagnosed muscle biopsies were examined by dry blood spot testing (DBS) including determination of the enzyme activity of acid alpha-glucosidase (GAA). In the case of depressed enzyme activity, additional gene testing of the GAA gene was carried out...
December 7, 2017: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/29325298/-a-case-of-glycogen-storage-disease-type-ii-and-related-analysis
#4
J M Han, L Y Zhang, L Sun, Y Lu, M H Li
No abstract text is available yet for this article.
December 20, 2017: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/29318410/flux-analysis-of-inborn-errors-of-metabolism
#5
D-J Reijngoud
Patients with an inborn error of metabolism (IEM) are deficient of an enzyme involved in metabolism, and as a consequence metabolism reprograms itself to reach a new steady state. This new steady state underlies the clinical phenotype associated with the deficiency. Hence, we need to know the flux of metabolites through the different metabolic pathways in this new steady state of the reprogrammed metabolism. Stable isotope technology is best suited to study this. In this review the progress made in characterizing the altered metabolism will be presented...
January 9, 2018: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/29312541/energy-metabolism-in-glioblastoma-stem-cells-ppar%C3%AE-a-metabolic-adaptor-to-intratumoral-microenvironment
#6
Alessia Fidoamore, Loredana Cristiano, Chiara Laezza, Renato Galzio, Elisabetta Benedetti, Benedetta Cinque, Andrea Antonosante, Michele d'Angelo, Vanessa Castelli, Maria Grazia Cifone, Rodolfo Ippoliti, Antonio Giordano, Annamaria Cimini
Glioblastoma (GB), the most-common cancer in the adult brain, despite surgery and radio/ chemotherapy, is to date almost incurable. Many hypoxic tumors, including GB, show metabolic reprogramming to sustain uncontrolled proliferation, hypoxic conditions and angiogenesis. Peroxisome Proliferator-activated Receptors (PPAR), particularly the α isotype, have been involved in the control of energetic metabolism. Herein, we characterized patient-derived GB neurospheres focusing on their energetic metabolism and PPARα expression...
December 12, 2017: Oncotarget
https://www.readbyqxmd.com/read/29311486/effect-of-seleno-l-methionine-on-oxidative-stress-in-the-pancreatic-islets-of-a-short-term-induced-diabetic-mouse-model-in-insufficient-selenium-status
#7
Hitoshi Ueno, Ryo Shimizu, Tomofumi Okuno, Hirofumi Ogino, Tomohiro Arakawa, Koichi Murano, Katsuhiko Nakamuro
The protective effects of seleno-L-methionine (SeMet) on oxidative stress in pancreatic islets were investigated with a short-term nicotinamide (NA) and streptozotocin (STZ)-induced diabetic mouse model. ICR mice were intraperitoneally injected twice with 100 mg/kg STZ and 120 mg/kg NA at a 1-d interval and were then orally administered 158 µg Se/kg SeMet with free access to a selenium-deficient diet for 5 weeks. Administration of SeMet significantly improved the levels of glycated hemoglobin (HbA1c), non-fasting and oral glucose tolerance-tested (OGTT) blood glucose, plasma adiponectin and hepatic glycogen that deteriorated by NA/STZ treatment...
2018: Biological & Pharmaceutical Bulletin
https://www.readbyqxmd.com/read/29302509/a-novel-homozygous-lipa-mutation-in-a-korean-child-with-lysosomal-acid-lipase-deficiency
#8
Kwang Yeon Kim, Ju Whi Kim, Kyung Jae Lee, Eunhyang Park, Gyeong Hoon Kang, Young Hun Choi, Woo Sun Kim, Jung Min Ko, Jin Soo Moon, Jae Sung Ko
Patients with lysosomal acid lipase (LAL) deficiency and glycogen storage disease (GSD) demonstrated hepatomegaly and dyslipidemia. In our case, a 6-year-old boy presented with hepatosplenomegaly. At 3 years of age, GSD had been diagnosed by liver biopsy at another hospital. He showed elevated serum liver enzymes and dyslipidemia. Liver biopsy revealed diffuse microvesicular fatty changes in hepatocytes, septal fibrosis and foamy macrophages. Ultrastructural examination demonstrated numerous lysosomes that contained lipid material and intracytoplasmic cholesterol clefts...
December 2017: Pediatric Gastroenterology, Hepatology & Nutrition
https://www.readbyqxmd.com/read/29298619/developing-a-cost-effective-and-scalable-production-of-human-hepatic-competent-endoderm-from-size-controlled-pluripotent-stem-cell-aggregates
#9
Zahra Farzaneh, Mostafa Najarasl, Saeed Abbasalizadeh, Massoud Vosough, Hossein Baharvand
Dynamic suspension culture of human pluripotent stem cells (hPSCs) in stirred bioreactors provides a valuable scalable culture platform for integrated differentiation toward different lineages for potential research and therapeutic applications. However, current protocols for scalable and integrated differentiation of hPSCs limited due to high cost of growth factors and technical challenges. Here, hPSCs aggregates primed with 6 and 12 µM of CHIR99021 (CHIR), a Wnt agonist, in combination with different concentrations of high cost Activin A (10, 25, 50, 100 ng/ml)...
January 3, 2018: Stem Cells and Development
https://www.readbyqxmd.com/read/29284117/knockdown-of-ampk%C3%AE-decreases-atm-expression-and-increases-radiosensitivity-under-hypoxia-and-nutrient-starvation-in-an-sv40-transformed-human-fibroblast-cell-line-lm217
#10
Yasuhiko Murata, Takuma Hashimoto, Yusuke Urushihara, Soichiro Shiga, Kazuya Takeda, Keiichi Jingu, Yoshio Hosoi
BACKGROUND: Presence of unperfused regions containing cells under hypoxia and nutrient starvation contributes to radioresistance in solid human tumors. It is well known that hypoxia causes cellular radioresistance, but little is known about the effects of nutrient starvation on radiosensitivity. We have reported that nutrient starvation induced decrease of mTORC1 activity and decrease of radiosensitivity in an SV40-transformed human fibroblast cell line, LM217, and that nutrient starvation induced increase of mTORC1 activity and increase of radiosensitivity in human liver cancer cell lines, HepG2 and HuH6 (Murata et al...
December 25, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/29240773/polypyridylruthenium-ii-complexes-exert-anti-schistosome-activity-and-inhibit-parasite-acetylcholinesterases
#11
Madhu K Sundaraneedi, Bemnet Tedla, Ramon M Eichenberger, Luke Becker, Darren Pickering, Michael J Smout, Siji Rajan, Phurpa Wangchuk, F Richard Keene, Alex Loukas, J Grant Collins, Mark S Pearson
BACKGROUND: Schistosomiasis affects over 200 million people and there are concerns whether the current chemotherapeutic control strategy (periodic mass drug administration with praziquantel (PZQ)-the only licenced anti-schistosome compound) is sustainable, necessitating the development of new drugs. METHODOLOGY/PRINCIPAL FINDINGS: We investigated the anti-schistosome efficacy of polypyridylruthenium(II) complexes and showed they were active against all intra-mammalian stages of S...
December 14, 2017: PLoS Neglected Tropical Diseases
https://www.readbyqxmd.com/read/29232314/postexercise-fructose-maltodextrin-ingestion-enhances-subsequent-endurance-capacity
#12
Ed Maunder, Tim Podlogar, Gareth A Wallis
PURPOSE: Restoring skeletal muscle and hepatic glycogen content during short-term (<6 h) recovery from prolonged exercise is pertinent for athletes seeking to maximize performance in repeated exercise bouts. Previous research suggests co-ingestion of fructose-glucose carbohydrate sources augments hepatic and has equivalent effects on skeletal muscle glycogen storage during short-term recovery from prolonged exercise compared to isocaloric glucose ingestion. The aim of the present investigation was to determine if this has a discernible effect on subsequent exercise capacity...
December 11, 2017: Medicine and Science in Sports and Exercise
https://www.readbyqxmd.com/read/29223626/sleep-and-quality-of-life-of-patients-with-glycogen-storage-disease-on-standard-and-modified-uncooked-cornstarch
#13
Isabelle Rousseau-Nepton, Céline Huot, Diane Laforte, Elise Mok, Daphna Fenyves, Evelyn Constantin, John Mitchell
BACKGROUND: Glycemic control in hepatic glycogen storage diseases (GSDs) relies on specific nutritional recommendations, including strict avoidance of a fasting period. Uncooked cornstarch (UCCS) is an important therapeutic component. A new modified UCCS, Glycosade™, was created with the objective of prolonging euglycemia. We aimed to determine the length of euglycemia on Glycosade™ using a continuous glucose monitor (CGM) and to evaluate whether longer euglycemia and thus less nighttime interruptions would improve sleep and quality of life (QoL) after the introduction of the modified cornstarch...
September 11, 2017: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/29218530/tracking-glut2-translocation-by-live-cell-imaging
#14
Sabina Tsytkin-Kirschenzweig, Merav Cohen, Yaakov Nahmias
The facilitative glucose transporter (GLUT) family plays a key role in metabolic homeostasis, controlling the absorption rates and rapid response to changing carbohydrate levels. The facilitative GLUT2 transporter is uniquely expressed in metabolic epithelial cells of the intestine, pancreas, liver, and kidney. GLUT2 dysfunction is associated with several pathologies, including Fanconi-Bickel syndrome, a glycogen storage disease, characterized by growth retardation and renal dysfunction. Interestingly, GLUT2 activity is modulated by its cellular localization...
2018: Methods in Molecular Biology
https://www.readbyqxmd.com/read/29215735/vacuolated-pas-positive-lymphocytes-as-an-hallmark-of-pompe-disease-and-other-myopathies-related-to-impaired-autophagy
#15
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/29203351/tamarindus-indica-seeds-improve-carbohydrate-and-lipid-metabolism-an-in%C3%A2-vivo-study
#16
Uzukwu Emmanuel Uchenna, Amal Bakr Shori, Ahmad Salihin Baba
BACKGROUND: The tamarind seeds have a lot of nutrients that may be used to control cholesterol or glucose levels. OBJECTIVES: The effects of tamarind seeds (T) on lipid and carbohydrate metabolism in rats were studied. Rats were offered basal diet (BD) with T (2%, 4% or 8%) or without T. MATERIALS AND METHODS: Feeding and growth performance in rats were measured and samples of liver and blood were analyzed for glycogen content and levels of cholesterol and glucose respectively...
December 1, 2017: Journal of Ayurveda and Integrative Medicine
https://www.readbyqxmd.com/read/29203241/the-splicing-factor-transformer2-tra2-functions-in-the-drosophila-fat-body-to-regulate-lipid-storage
#17
Cezary Mikoluk, Alexis A Nagengast, Justin R DiAngelo
Excess nutrients are stored as triglycerides mainly in the adipose tissue of an animal and these triglycerides are located in structures called lipid droplets. Previous genome-wide RNAi screens in Drosophila cells identified splicing factors as playing a role in lipid droplet formation. Our lab has recently identified the SR protein, 9G8, as an important factor in fat storage as decreasing its levels results in augmented triglyceride storage in the fat body. Previous in vitro studies have implicated 9G8 in the regulation of splicing of the sex determination gene doublesex (dsx) by binding to transformer (tra) and transformer2 (tra2); however, any function of these sex determination proteins in regulating metabolism is unknown...
December 1, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/29202537/-the-clinicopathological-characteristics-and-genetic-analysis-of-a-case-of-late-onset-glycogen-storage-disease-type-%C3%A2-caused-by-a-novel-deletion-mutation
#18
A P Sun, X Y Liu, Y S Zhang
No abstract text is available yet for this article.
December 1, 2017: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
https://www.readbyqxmd.com/read/29200956/stem-cells-from-cryopreserved-human-dental-pulp-tissues-sequentially-differentiate-into-definitive-endoderm-and-hepatocyte-like-cells-in-vitro
#19
Young-Jin Han, Young-Hoon Kang, Sarath Belame Shivakumar, Dinesh Bharti, Young-Bum Son, Yong-Ho Choi, Won-Uk Park, June-Ho Byun, Gyu-Jin Rho, Bong-Wook Park
We previously described a novel tissue cryopreservation protocol to enable the safe preservation of various autologous stem cell sources. The present study characterized the stem cells derived from long-term cryopreserved dental pulp tissues (hDPSCs-cryo) and analyzed their differentiation into definitive endoderm (DE) and hepatocyte-like cells (HLCs) in vitro. Human dental pulp tissues from extracted wisdom teeth were cryopreserved as per a slow freezing tissue cryopreservation protocol for at least a year...
2017: International Journal of Medical Sciences
https://www.readbyqxmd.com/read/29187643/rescue-of-pompe-disease-in-mice-by-aav-mediated-liver-delivery-of-secretable-acid-%C3%AE-glucosidase
#20
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
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