Showing 1-30 of 102 results for "

g1767

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Fiore Manganelli et al.
Acta myologica : myopathies and cardiomyopathies : official journal of the Mediterranean Society of Myology, 32(2), 82-84 (2014-01-09)
Glycogen storage disease type II - also called Pompe disease or acid maltase deficiency - is an autosomal recessive metabolic disorder, caused by an accumulation of glycogen in the lysosome due to deficiency of the lysosomal acid alpha-glucosidase enzyme. Pompe...
Dang Hai Dang Nguyen et al.
Journal of bacteriology, 196(11), 1941-1949 (2014-03-13)
We studied the activity of a debranching enzyme (TreX) from Sulfolobus solfataricus on glycogen-mimic substrates, branched maltotetraosyl-β-cyclodextrin (Glc₄-β-CD), and natural glycogen to better understand substrate transglycosylation and the effect thereof on glycogen debranching in microorganisms. The validation test of Glc₄-β-CD...
Bridget E Lester et al.
Aviation, space, and environmental medicine, 84(8), 789-796 (2013-08-10)
Studies of real and simulated microgravity exposure show the lower limb muscles atrophy to the greatest extent, with the calf muscles being most affected and most difficult to target with exercise countermeasures. This ground-based study examined the metabolic involvement of...
Timothy J Sullivan et al.
Metabolism: clinical and experimental, 62(11), 1623-1632 (2013-08-21)
CCR2 inhibition has produced promising experimental and clinical anti-hyperglycemic effects. These results support the thesis that insulin resistance and Type 2 diabetes (T2D) are associated with chronic unresolved inflammation. The aim of this study was to provide a broad analysis...
Biomass composition of blue mussels, Mytilus edulis, is affected by living site and species of ingested microalgae
Pleissner D, et al.
ISRN zoology, 2012 (2012)
Qi Zhang et al.
Journal of chromatography. A, 1306, 97-103 (2013-08-06)
In this paper, two novel amino acid chiral ILs, tetramethylammonium-l-arginine (TMA-l-Arg) and tetramethylammonium-l-aspartic acid (TMA-l-Asp), were applied for the first time in CE enantioseparation to evaluate their potential synergistic effect with glycogen as chiral selector. As observed, significantly improved separation...
Benjamin L Farah et al.
FASEB journal : official publication of the Federation of American Societies for Experimental Biology, 28(5), 2272-2280 (2014-01-23)
Enzyme or gene replacement therapy with acid α-glucosidase (GAA) has achieved only partial efficacy in Pompe disease. We evaluated the effect of adjunctive clenbuterol treatment on cation-independent mannose-6-phosphate receptor (CI-MPR)-mediated uptake and intracellular trafficking of GAA during muscle-specific GAA expression...
Alberto Parras et al.
Nature, 560(7719), 441-446 (2018-08-17)
Common genetic contributions to autism spectrum disorder (ASD) reside in risk gene variants that individually have minimal effect sizes. As environmental factors that perturb neurodevelopment also underlie idiopathic ASD, it is crucial to identify altered regulators that can orchestrate multiple...
Lilijana Bizjak Mali et al.
Journal of morphology, 274(8), 887-900 (2013-04-30)
The morphological alterations of hepatocytes of cave-dwelling salamander Proteus anguinus anguinus after food deprivation periods of one and 18 months were investigated and the concentrations of glycogen, lipids, and proteins in the liver were determined. Quantitative analyses of the hepatocyte...
Hyo-Jin Jeong et al.
Biochemical and biophysical research communications, 434(3), 503-508 (2013-04-16)
MicroRNA (miRNA) is a class of endogenous small noncoding RNA that negatively regulates gene expression at the post-transcriptional level and plays an important role in the pathogenesis of various diseases. However, the identity and role of miRNAs involved in the...
Malgorzata Gasperowicz et al.
Developmental biology, 382(1), 1-14 (2013-08-21)
TLE3 is a transcriptional co-repressor that interacts with several DNA-binding repressors, including downstream effectors of the Notch signaling pathway. We generated Tle3-deficient mice and found that they die in utero and their death is associated with abnormal development of the...
E Fitzpatrick et al.
Archives of disease in childhood, 99(4), 354-357 (2014-01-15)
Mauriac syndrome is characterised by growth failure, cushingoid appearance and hepatomegaly which occurs in patients with insulin dependent diabetes and was first described shortly after the introduction of insulin as a treatment for the condition. To describe the clinical features...
A A Zhidenko et al.
Ukrains'kyi biokhimichnyi zhurnal (1999 ), 85(3), 22-30 (2013-08-14)
The use of glyphosate as a herbicide in agriculture can lead to the presence of its residues and metabolites (aminomethylphosphonic acid) in food for human consumption and pose a threat to health. The effect of these herbicides on the fish...
Angelika Hartung et al.
Biochimica et biophysica acta, 1833(12), 2803-2811 (2013-07-28)
Akt kinases are important mediators of the insulin signal, and some Akt substrates are directly involved in glucose homeostasis. Recently, Girdin has been described as an Akt substrate that is expressed ubiquitously in mammals. Cells overexpressing Girdin show an enhanced...
Nicholas E E Allenby et al.
Nucleic acids research, 40(19), 9543-9556 (2012-08-21)
Streptomycetes sense and respond to the stress of phosphate starvation via the two-component PhoR-PhoP signal transduction system. To identify the in vivo targets of PhoP we have undertaken a chromatin-immunoprecipitation-on-microarray analysis of wild-type and phoP mutant cultures and, in parallel...
Robyn G Lottes et al.
American journal of physiology. Lung cellular and molecular physiology, 306(10), L947-L955 (2014-04-01)
Although many lung diseases are associated with hypoxia, alveolar type II epithelial (ATII) cell impairment, and pulmonary surfactant dysfunction, the effects of O(2) limitation on metabolic pathways necessary to maintain cellular energy in ATII cells have not been studied extensively....
Suppression of autophagic activation in the mouse uterus by estrogen and progesterone.
Choi S, Shin H, Song H, et al.
The Journal of Endocrinology, 221(1), 39-50 (2014)
Lisa C Heather et al.
Circulation. Heart failure, 6(5), 1058-1066 (2013-08-14)
Fatty acid and glucose transporters translocate between the sarcolemma and intracellular compartments to regulate substrate metabolism acutely. We hypothesised that during ischemia fatty acid translocase (FAT/CD36) would translocate away from the sarcolemma to limit fatty acid uptake when fatty acid...
Juan M Pascual et al.
JAMA neurology, 70(6), 756-763 (2013-04-24)
The physiological relevance of acid maltase (acid α-glucosidase, an enzyme that degrades lysosomal glycogen) is well recognized in liver and muscle. In late (adult)-onset acid maltase deficiency (glycogen storage disease type II [GSD II]), glycogen accumulates inside muscular lysosomes in...
Valentina Gburcik et al.
American journal of physiology. Endocrinology and metabolism, 304(8), E885-E894 (2013-03-14)
Failure of white adipose tissue to appropriately store excess metabolic substrate seems to underpin obesity-associated type 2 diabetes. Encouraging "browning" of white adipose has been suggested as a therapeutic strategy to help dispose of excess stored lipid and ameliorate the...
Joachim Nielsen et al.
Applied physiology, nutrition, and metabolism = Physiologie appliquee, nutrition et metabolisme, 38(2), 91-99 (2013-02-27)
Glucose is stored in skeletal muscle fibers as glycogen, a branched-chain polymer observed in electron microscopy images as roughly spherical particles (known as β-particles of 10-45 nm in diameter), which are distributed in distinct localizations within the myofibers and are...
Neeraj Verma et al.
Indian journal of experimental biology, 51(1), 65-72 (2013-02-28)
Antihyperglycemic potential of hyperin at 25 and 50 mg/kg doses for 30 days to streptozotocin induced diabetic rats has been reported. In oral glucose tolerance test, hyperin treated rats showed a significant reduction in blood glucose level after 120 min....
Francis B Stephens et al.
Clinical science (London, England : 1979), 127(5), 315-322 (2014-03-13)
Acylcarnitine accumulation in skeletal muscle and plasma has been observed in numerous models of mitochondrial lipid overload and insulin resistance. Fish oil n3PUFA (omega-3 polyunsaturated fatty acids) are thought to protect against lipid-induced insulin resistance. The present study tested the...
Rathishri Chandurvelan et al.
Aquatic toxicology (Amsterdam, Netherlands), 140-141, 303-313 (2013-07-24)
The biochemical responses of the green-lipped mussel, Perna canaliculus, to waterborne cadmium (Cd) were investigated in order to delineate toxic mechanisms, and the impacts of exposure dose and duration, of this important toxicant in a potential sentinel species. Mussels were...
Massimiliano Filosto et al.
Acta myologica : myopathies and cardiomyopathies : official journal of the Mediterranean Society of Myology, 32(2), 91-94 (2014-01-09)
Glycogenosis II (GSD II) is an autosomal recessive lysosomal storage disorder resulting from acid alpha-glucosidase deficiency, subsequent accumulation of glycogen in tissues, impairment of autophagic processes and progressive cardiac, motor and respiratory failure. The late-onset form is characterized by wide...
Ahmed Th A Ibrahim et al.
Ecotoxicology and environmental safety, 106, 33-39 (2014-05-20)
The present study examined the impacts of carbofuran on endocrinology of the catfish, Clarias gariepinus, for the first time and evaluated cortisol (CRT), triiodothyronine (T3), thyroxin (T4), 17β-estradiol (E2) and testosterone (TST) and the oxidative stress markers including SOD, CAT...
Eric A F Herbst et al.
American journal of physiology. Regulatory, integrative and comparative physiology, 306(2), R102-R107 (2013-12-07)
During recovery from glycogen-depleting exercise, there is a shift from carbohydrate oxidation to glycogen resynthesis. The activity of the pyruvate dehydrogenase (PDH) complex may decrease to reduce oxidation of carbohydrates in favor of increasing gluconeogenic recycling of carbohydrate-derived substrates for...
Gustavo Gomes de Araujo et al.
Laboratory animals, 47(1), 36-42 (2013-03-08)
This study was undertaken to characterize the effects of monotonous training at lactate minimum (LM) intensity on aerobic and anaerobic performances; glycogen concentrations in the soleus muscle, the gastrocnemius muscle and the liver; and creatine kinase (CK), free fatty acids...
M José Villarroel et al.
Environmental toxicology and pharmacology, 35(3), 465-472 (2013-03-19)
The present study investigates energy stores changes in the aquatic invertebrate Daphnia magna following a 5-d exposure to propanil. Juveniles of D. magna were exposed to sublethal propanil concentrations (0.07, 0.10, 0.21 and 0.55 mgl(-1)) which were used previously to...
Carlos M Castorena et al.
Diabetes, 63(7), 2297-2308 (2014-03-13)
Earlier research on rats with normal insulin sensitivity demonstrated that acute exercise increased insulin-stimulated glucose uptake (GU) concomitant with greater phosphorylation of Akt substrate of 160 kDa (pAS160). Because mechanisms for exercise effects on GU in insulin-resistant muscle are unknown...