Results 11 to 20 of about 337,150 (208)

Liver transplantation for type IV glycogen storage disease [PDF]

, 1991
TYPE IV glycogen storage disease is a rare autosomal recessive disorder (also called Andersen's disease1 or amylopectinosis) in which the activity of branching enzyme alpha-1, 4-glucan: alpha-1, 4-glucan 6-glucosyltransferase is deficient in the liver as
Andreas Tzakis, Bannayan, Barbara I. Brown, Brown, Eduardo Yunis, Greene, Illingworth, Ishihara, Ishihara, Mazzaferro, McMaster, Reed, Rick Selby, Ross S. Kendall, Schochet, Stand, Starzl, Thomas E. Starzl   +17 more
core   +1 more source

Glycogen Metabolism in Glycogen-rich Erythrocytes [PDF]

Blood, 1974
Abstract High concentrations of red blood cell glycogen were visualized by electron microscopy and demonstrated biochemically in amylo-1,6-glucosidase- and phosphorylase-deficient red blood cells. Glycogen concentration decreased as a function of cell age.
S W, Moses, N, Bashan, A, Gutman, P A, Ockerman   +3 more
openaire   +2 more sources

Crosstalk between beta‐adrenergic and insulin signaling mediates mechanistic target of rapamycin hyperactivation in liver of high‐fat diet‐fed male mice

Physiological Reports, 2021
Nonalcoholic fatty liver disease (NAFLD) is the most common cause of chronic liver disease. While increased nutrient intake and sympathetic activity have been associated with the disease, the pathogenesis of NAFLD remains incompletely understood.
Sadia Ashraf, Nadia Ashraf, Gizem Yilmaz, Romain Harmancey   +3 more
doaj   +1 more source

Lafora disease offers a unique window into neuronal glycogen metabolism [PDF]

, 2018
Lafora disease (LD) is a fatal, autosomal recessive, glycogen-storage disorder that manifests as severe epilepsy. LD results from mutations in the gene encoding either the glycogen phosphatase laforin or the E3 ubiquitin ligase malin. Individuals with LD
Gentry, Matthew S., Guinovart, Joan J., Minassian, Berge A., Roach, Peter J., Serratosa, Jose M.   +4 more
core   +2 more sources

Liver Glycogen Phosphorylase Deficiency Leads to Profibrogenic Phenotype in a Murine Model of Glycogen Storage Disease Type VI

Hepatology Communications, 2019
Mutations in the liver glycogen phosphorylase (Pygl) gene are associated with the diagnosis of glycogen storage disease type VI (GSD‐VI). To understand the pathogenesis of GSD‐VI, we generated a mouse model with Pygl deficiency (Pygl−/−).
Lane H. Wilson, Jun‐Ho Cho, Ana Estrella, Joan A. Smyth, Rong Wu, Tayoot Chengsupanimit, Laurie M. Brown, David A. Weinstein, Young Mok Lee   +8 more
doaj   +1 more source

Glycogen metabolism and glycogen storage disorders [PDF]

Annals of Translational Medicine, 2018
Glucose is the main energy fuel for the human brain. Maintenance of glucose homeostasis is therefore, crucial to meet cellular energy demands in both - normal physiological states and during stress or increased demands. Glucose is stored as glycogen primarily in the liver and skeletal muscle with a small amount stored in the brain.
Shibani, Kanungo, Kimberly, Wells, Taylor, Tribett, Areeg, El-Gharbawy   +3 more
openaire   +2 more sources

A forage-only diet alters the metabolic response of horses in training [PDF]

, 2012
Most athletic horses are fed a high-starch diet despite the risk of health problems. Replacing starch concentrate with high-energy forage would alleviate these health problems, but could result in a shift in major substrates for muscle energy supply from
A. Jansson, Connysson, Connysson, Danielsen, Devlin, Ellis, Essén-Gustavsson, Essén-Gustavsson, Farris, Geor, Gillham, Glade, Harris, Hintz, Houpt, Hudson, Hyyppä, J.E. Lindberg, Kelso, Lacombe, Lacombe, Lawrence, Lindholm, Lowry, Luthersson, MacLeay, Meyer, Muhonen, Pagan, Palmgren-Karlsson, Palmgren-Karlsson, Persson, Potter, Ragnarsson, Redbo, Schuback, Snow, Tinker, Voiton, Waller, Waller, Waters, Willard, Williamson, Willing, Wilson   +45 more
core   +2 more sources

Metabolic crosstalk: molecular links between glycogen and lipid metabolism in obesity. [PDF]

, 2014
Glycogen and lipids are major storage forms of energy that are tightly regulated by hormones and metabolic signals. We demonstrate that feeding mice a high-fat diet (HFD) increases hepatic glycogen due to increased expression of the glycogenic ...
Bridges, Dave, Chang, Louise, Cheng, Alan, Downes, Michael, Evans, Ronald M, Fisher, Kaleigh, Liddle, Christopher, Lin, Jiandie D, Lu, Binbin, Meng, Zhuo-Xian, Saltiel, Alan R, Yang, Yemen, Yu, Ruth T   +12 more
core   +2 more sources

AAV-mediated transcription factor EB (TFEB) gene delivery ameliorates muscle pathology and function in the murine model of Pompe Disease [PDF]

, 2017
Pompe disease (PD) is a metabolic myopathy due to acid alpha-glucosidase deficiency and characterized by extensive glycogen storage and impaired autophagy.
Alvino, Filomena Grazia, Auricchio, Alberto, Ballabio, Andrea, Carrella, Alessandra, De Leonibus, Elvira, Diez-roux, Graciana, Gatto, Francesca, Iacobellis, Francesca, Nusco, Edoardo, Parenti, Giancarlo, Polishchuk, Elena, Polishchuk, Roman, Rossi, Barbara, Tarallo, Antonietta   +13 more
core   +1 more source

Pre-implantation mouse embryos cultured In vitro under different oxygen concentrations show altered ultrastructures [PDF]

, 2020
Assisted Reproductive Technologies routinely utilize different culture media and oxygen (O2) concentrations to culture human embryos. Overall, embryos cultured under physiological O2 tension (5%) have improved development compared to embryos cultured ...
Antonouli, S, Belli, M, Macchiarelli, G, Nottola, Sa, Palmerini, Mg, Rinaudo, P, Ruggeri, E   +6 more
core   +1 more source