Results 71 to 80 of about 37,247 (212)
Epigallocatechin‐3‐gallate (EGCG) acutely inhibited gluconeogenesis and enhanced glycolysis, glycogenolysis, and fatty acid oxidation in perfused rat livers. Mechanistic assays revealed mitochondrial uncoupling, inhibition of pyruvate carboxylation and glucose‐6‐phosphatase, shift of NADH/NAD+ ratios toward oxidation, and loss of membrane integrity ...
Carla Indianara Bonetti +8 more
wiley +1 more source
Glycogen Storage Disease in Twins: When Two Lives Reflect One Silent Battle
ABSTRACT Early recognition of hepatomegaly, hypoglycemia, and elevated liver enzymes in infants is crucial for diagnosing glycogen storage disease. Liver biopsy with PAS–diastase staining provides definitive confirmation. Prompt metabolic management and regular follow‐up are essential to prevent progression of fibrosis and long‐term hepatic ...
Rajat Kumar Shah +10 more
wiley +1 more source
Efzimfotase Alfa Improves Respiratory Capacity in Muscle Tissue From a Mouse Model of HPP
ABSTRACT Hypophosphatasia (HPP) is an inherited metabolic disease caused by deficient tissue‐nonspecific alkaline phosphatase (ALP) activity and characterized by skeletal and nonskeletal symptoms, including muscle weakness and fatigue. We hypothesized that mitochondrial respiration is impaired in muscle in HPP, independent of skeletal manifestations ...
Denise Devore +10 more
wiley +1 more source
Experiments were designed to study in-vivo effects of sodium cyanide on biochemical endpoints in the freshwater fish Labeo rohita. Fish were exposed to two sublethal concentrations (0.106 and 0.064mg/L) for a period of 15 days.
Praveen N. Dube +2 more
doaj +1 more source
50th anniversary of the Stanford SSRL synchrotron radiation and protein crystallography initiative
We provide a historical introduction and our thoughts on the current trends including some based on papers in this special issue of Journal of Synchrotron Radiation celebrating the 50th Anniversary of the Stanford SSRL synchrotron radiation and protein crystallography initiative led by Keith Hodgson.We provide a historical introduction spanning the ...
John R. Helliwell +2 more
wiley +1 more source
McArdle Disease and Exercise Physiology
McArdle disease (glycogen storage disease Type V; MD) is a metabolic myopathy caused by a deficiency in muscle glycogen phosphorylase. Since muscle glycogen is an important fuel for muscle during exercise, this inborn error of metabolism provides a model
Yu Kitaoka
doaj +1 more source
Extracellular vesicles (EVs) are tiny, membrane‐covered packets that cells naturally release to send molecular information to one another. In the brain, they function as couriers that move proteins, lipids, and other signals between neurons, glial cells, and the blood–brain barrier.
Berenice N. Bernal‐Vicente +4 more
wiley +1 more source
Dietary Management of the Ketogenic Glycogen Storage Diseases
The glycogen storage diseases (GSDs) comprise a group of rare inherited disorders of glycogen metabolism. The hepatic glycogenolytic forms of these disorders are typically associated with hypoglycemia and hepatomegaly.
Kaustuv Bhattacharya MBBS, MRCPCH, FRACP, MD +2 more
doaj +1 more source
LIVER TRANSPLANTATION FOR TYPE I GLYCOGEN STORAGE DISEASE [PDF]
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 +15 more
core +1 more source

