Results 41 to 50 of about 337,150 (208)

LIVER TRANSPLANTATION FOR TYPE I GLYCOGEN STORAGE DISEASE [PDF]

, 1983
A 16½-year-old girl with type I glycogen storage disease was treated by orthotopic liver transplantation under cyclosporin/steroid immunosuppression. All metabolic stigmata of the disease were relieved and 1 year postoperatively she follows a normal diet
Alper, Alper Ca, Con, Folkman, Greene, Greene, Kashiwagi, Kashiwagi, Merrill, Nordlie, Raum, Starzl, Starzl, Starzl, Starzl, Starzl   +15 more
core   +1 more source

Short and long-term acceptability and efficacy of extended-release cornstarch in the hepatic glycogen storage diseases: results from the Glyde study

Orphanet Journal of Rare Diseases
Background Hypoglycaemia is the primary manifestation of all the hepatic types of glycogen storage disease (GSD). In 2008, Glycosade®, an extended-release waxy maize cornstarch, was reported as an alternative to uncooked cornstarch (UCCS) which could ...
DA Weinstein, RJ Jackson, EA Brennan, M Williams, JE Davison, F de Boer, TGJ Derks, C Ellerton, B Faragher, J Gribben, P Labrune, KM McKittrick, E Murphy , KM Ross, U Steuerwald, C Voillot, AJM Woodward, HR Mundy   +17 more
doaj   +1 more source

Influence of hypoxia on biochemical aspects and on expression of genes related to oxygen-homeostasis of the Amazonian cichlid Astronotus ocellatus (Agassiz, 1831)

Genetics and Molecular Biology, 2021
Variations in dissolved oxygen levels are common in the Amazonian aquatic environments and the aquatic organisms that inhabit these environments developed a variety of adaptive responses to deal with such conditions.
José L. Vasconcelos-Lima, Vanessa L. Oikawa-Cardoso, Waldir Heinrichs-Caldas, Vera M. F. Almeida-Val   +3 more
doaj   +1 more source

Physicochemical and Spectroscopic Characterization of Glycogen and Glycogen Phosphorylase b Complexes

Polysaccharides
Glycogen is a natural polysaccharide used as an energy storage macromolecule. The role of glycogen metabolism in type 2 diabetes mellitus has been under investigation for several years, along with its implication in cancer and cardiovascular and ...
Pandora Karakousi, Maria Karayianni, Evangelia D. Chrysina, Stergios Pispas   +3 more
doaj   +1 more source

Effect of starvation and subsequent feeding on glycogen concentration, behavior and mortality in the golden mussel Limnoperna fortunei (Dunker, 1857) (Bivalvia: Mytilidae

Journal of Limnology, 2016
The success of Limnoperna fortunei as an invasive species is related to its physiological plasticity that allows them to endure adverse environmental conditions.
Nelmara I.S. Cordeiro, Jennifer T.M. Andrade, Lângia C. Montresor, Dalva M.R. Luz, Carlos B. Martinez, Gustavo Darrigran, Jairo Pinheiro, Teofânia H.D.A. Vidigal   +7 more
doaj   +1 more source

From glycogen metabolism to Lafora disease [PDF]

, 2016
Glycogen synthesis is normally absent in neurons. However, inclusion bodies resembling abnormal glycogen accumulate in several neurological diseases, particularly in progressive myoclonus epilepsy or Lafora disease.
Guinovart, Joan
core  

The Autophagic Activator GHF-201 Can Alleviate Pathology in a Mouse Model and in Patient Fibroblasts of Type III Glycogenosis

Biomolecules
Glycogen storage disease type III (GSDIII) is a hereditary glycogenosis caused by deficiency of the glycogen debranching enzyme (GDE), an enzyme, encoded by Agl, enabling glycogen degradation by catalyzing alpha-1,4-oligosaccharide side chain transfer ...
Kumudesh Mishra, Sahar Sweetat, Saja Baraghithy, Uri Sprecher, Monzer Marisat, Sultan Bastu, Hava Glickstein, Joseph Tam, Hanna Rosenmann, Miguel Weil, Edoardo Malfatti, Or Kakhlon   +11 more
doaj   +1 more source

Glycogen Metabolism and Its Role in Growth and Encystation in Entamoeba histolytica

Life, 2023
Entamoeba histolytica is a parasitic protozoan that causes diarrheal disease in approximately 100 million people worldwide every year. E. histolytica has two forms, the growing trophozoite and the infectious cyst.
Jordan Wesel, Cheryl Ingram-Smith
doaj   +1 more source

Mixing unfamiliar lambs upon arrival at the abattoir affects their social behavior and meat traits

Frontiers in Animal Science
The aim of the study was to determine the effects of mixing unfamiliar lambs when they arrive at the abattoir 28-29 h before slaughtering on social behavior and meat quality. Forty Texel x Corriedale male lambs were transported together in the same truck
Javier Ithurralde, Rodolfo Ungerfeld, Geisibel Aparecida De Mello Maurina, Giuliano Moraes Figueiró, María Cristina Cabrera, Aline Freitas-de-Melo   +5 more
doaj   +1 more source

Metabolic Control, Quality of Life, and Body Image in Patients with Glycogen Storage Disease Type Ia [PDF]

, 2019
Glycogen storage disease is a group of inborn errors of metabolism, with type Ia being the most common form of the disorder. Glycogen storage disease type Ia (GSDIa) is a multisystemic condition in which individuals have various complications secondary ...
Bream, Alexa
core   +1 more source