Results 61 to 70 of about 2,890 (187)

Data from Loss of Glycogen Debranching Enzyme AGL Drives Bladder Tumor Growth via Induction of Hyaluronic Acid Synthesis

, 2023
Sunny Guin, Yuanbin Ru, Neeraj Agarwal, Carolyn R. Lew, Charles Owens, Giacomo P. Comi, Dan Theodorescu   +6 more
openalex   +2 more sources

Analysis of carbohydrates and glycoconjugates by matrix‐assisted laser desorption/ionization mass spectrometry: An update for 2021–2022

Mass Spectrometry Reviews, Volume 44, Issue 3, Page 213-453, May/June 2025.
Abstract The use of matrix‐assisted laser desorption/ionization (MALDI) mass spectrometry for the analysis of carbohydrates and glycoconjugates is a well‐established technique and this review is the 12th update of the original article published in 1999 and brings coverage of the literature to the end of 2022.
David J. Harvey
wiley   +1 more source

Ketogenic diet treatment in adults with glycogenosis type IIIa (Morbus Cori)

Clinical Nutrition Experimental, 2019
Summary: Background: Glycogen storage disease type IIIa (GSDIIIa) is a defect of the debranching enzyme (amylo-1,6-glucosidase) in glycogenolysis and has an effect on the muscles and the liver.
Tobias Fischer, Helen Njoroge, Ulrike Och, Ira Klawon, Thorsten Marquardt   +4 more
doaj   +1 more source

Activity of porcine muscle glycogen debranching enzyme in relation to pH and temperature [PDF]

Meat Science, 2005
The activity of glycogen debranching enzyme (GDE) was studied in relation to pH value and temperature in porcine masseter and longissimus dorsi muscles. A glycogen limit dextrin was used as the substrate for GDE, and the enzyme was derived from raw meat extracts. In both muscles, the pH only weakly affected the activity of GDE at the pH values found in
Maria, Kylä-Puhju, Marita, Ruusunen, Eero, Puolanne   +2 more
openaire   +2 more sources

From Stress to Success: Strategies for Improving Heat Tolerance in Wheat

Journal of Agronomy and Crop Science, Volume 211, Issue 3, May 2025.
ABSTRACT Wheat, a major staple crop, is critical for global food security. However, abiotic stresses, particularly heat stress, threaten crop productivity. With climate change predicted to increase temperatures by around 1.5°C by 2050, crop productivity could be severely affected.
Muhammad Ahmad, Maryium Sajjad, Aman Ullah, Usman Zulfiqar, Sami Ul‐Allah, Ejaz Ahmad Waraich, Kaleem ul Din, Kadambot H. M. Siddique, Muhammad Farooq   +8 more
wiley   +1 more source

Characteristics of metabolic myopathies among Egyptian patients

The Egyptian Journal of Neurology, Psychiatry and Neurosurgery
Background Metabolic Myopathies are characterized by defect in muscle energy production. The main symptoms are exercise intolerance, fatigue, myalgias, rhabdomyolysis and \or weakness, they present with episodic or fixed manifestations.
Mennat-Allah M. Elgamal, Hebatallah Rashed, Maha Nada, Hossam Sakr, Nagia Fahmy   +4 more
doaj   +1 more source

Deep morphological analysis of muscle biopsies from type III glycogenesis (GSDIII), debranching enzyme deficiency, revealed stereotyped vacuolar myopathy and autophagy impairment

Acta Neuropathologica Communications, 2019
Glycogen storage disorder type III (GSDIII), or debranching enzyme (GDE) deficiency, is a rare metabolic disorder characterized by variable liver, cardiac, and skeletal muscle involvement.
Pascal Laforêt, Michio Inoue, Evelyne Goillot, Claire Lefeuvre, Umut Cagin, Nathalie Streichenberger, Sarah Leonard-Louis, Guy Brochier, Angeline Madelaine, Clemence Labasse, Carola Hedberg-Oldfors, Thomas Krag, Louisa Jauze, Julien Fabregue, Philippe Labrune, Jose Milisenda, Aleksandra Nadaj-Pakleza, Sabrina Sacconi, Federico Mingozzi, Giuseppe Ronzitti, François Petit, Benedikt Schoser, Anders Oldfors, John Vissing, Norma B. Romero, Ichizo Nishino, Edoardo Malfatti   +26 more
doaj   +1 more source

mRNA therapy: A new frontier in regenerative medicine

Interdisciplinary Medicine, Volume 3, Issue 2, March 2025.
This review discussed the potential of mRNA drugs in regenearative medicine, highlighting the key processes of mRNA therapy, focusing on therapeutic mRNA modification and delivery carriers. The preclinical and clinical studies of mRNA therapeutics for regeneration of cardiac, lung, liver, kidney, locomotor system, skin lesions and neurological ...
Ding‐Ding Xue, Yue Zhang, An‐Ran Shen, Tao‐Tao Tang, Bi‐Cheng Liu, Lin‐Li Lv   +5 more
wiley   +1 more source

Heterozygous GAA knockout is nonconsequential on metabolism and the spatial liver transcriptome in high‐fat diet‐induced obese and prediabetic mice

Physiological Reports, Volume 13, Issue 6, March 2025.
Abstract Glycophagy is the autophagic degradation of glycogen by the enzyme acid alpha‐glucosidase (GAA). Although GAA inhibitors improve metabolic health by inhibiting GAA in the intestine, it is not clear if GAA inhibition in peripheral tissues such as the liver is metabolically beneficial. This study tested if the heterozygous knockout of GAA (HetKO‐
Cameron P. McCall, Melina C. Mancini, Jaroslaw Staszkiewicz, Douglas G. Mashek, Timothy D. Heden   +4 more
wiley   +1 more source

A Novel Nonsense Mutation of the AGL Gene in a Romanian Patient with Glycogen Storage Disease Type IIIa

Case Reports in Genetics, 2016
Background. Glycogen storage disease type III (GSDIII) is a rare metabolic disorder with autosomal recessive inheritance, caused by deficiency of the glycogen debranching enzyme.
Anca Zimmermann, Heidi Rossmann, Simona Bucerzan, Paula Grigorescu-Sido   +3 more
doaj   +1 more source