Results 81 to 90 of about 65,054 (194)

Medium-Chain Triglyceride Oil and Dietary Intervention Improved Body Composition and Metabolic Parameters in Children with Glycogen Storage Disease Type 1 in Jordan: A Clinical Trial

Foods
Glycogen storage diseases (GSDs) are a group of carbohydrate metabolism disorders, most of which are inherited in autosomal recessive patterns. GSDs are of two types: those that have to do with liver and hypoglycaemia (hepatic GSDs) and those that are ...
Hadil S. Subih, Reem A. Qudah, Sana Janakat, Hanadi Rimawi, Nour Amin Elsahoryi, Linda Alyahya   +5 more
doaj   +1 more source

Childhood Type II Diabetes: The Risks and Complications [PDF]

, 2015
Type II Diabetes Mellitus is an endocrine disorder that affects people of all ages. Type II Diabetes was once considered adult-onset diabetes, as it was nearly exclusively diagnosed in adults. Over the last three decades, the number of children diagnosed
Kapke, Danielle R
core   +1 more source

The thermodynamics of metabolism, cardiovascular performance and exercise, in health and diabetes: The objective of clinical markers [PDF]

, 2013
Extensive experience in UK National Health Service metabolic syndrome/type 2 diabetes clinics highlights the need for convenient clinical marker(s) which can be readily used to indicate the success or otherwise of alternative therapies.
Atherton, MA, Collins, MW, González-Alonso, J, König, CS, Ramachandran, S   +4 more
core  

Hepatic ChREBP orchestrates intrahepatic carbohydrate metabolism to limit hepatic glucose 6-phosphate and glycogen accumulation in a mouse model for acute Glycogen Storage Disease type Ib

Molecular Metabolism
Objective: Carbohydrate Response Element Binding Protein (ChREBP) is a glucose 6-phosphate (G6P)-sensitive transcription factor that acts as a metabolic switch to maintain intracellular glucose and phosphate homeostasis.
K.A. Krishnamurthy, M.G.S. Rutten, J.A. Hoogerland, T.H. van Dijk, T. Bos, M. Koehorst, M.P. de Vries, N.J. Kloosterhuis, H. Havinga, B.V. Schomakers, M. van Weeghel, J.C. Wolters, B.M. Bakker, M.H. Oosterveer   +13 more
doaj   +1 more source

Glycogen Storage Disease Type I [PDF]

, 2020
openaire   +1 more source

Natural history study of hepatic glycogen storage disease type IV and comparison to Gbe1ys/ys model

JCI Insight
Background Glycogen storage disease type IV (GSD IV) is an ultrarare autosomal recessive disorder that causes deficiency of functional glycogen branching enzyme and formation of abnormally structured glycogen termed polyglucosan. GSD IV has traditionally
Rebecca L. Koch, Bridget T. Kiely, Su Jin Choi, William R. Jeck, Leticia S. Flores, Vikrant Sood, Seema Alam, Gilda Porta, Katy LaVecchio, Claudia Soler-Alfonso, Priya S. Kishnani   +10 more
doaj   +1 more source

Polysaccharide storage myopathy - the story so far [PDF]

, 2015
Annandale, Annandale, Arad, Baird, Borgia, Borgia, Byrne, De La Corte, De La Corte, De La Corte, DiMauro, Firshman, Firshman, Firshman, Herszberg, Hunt, Johlig, Lacombe, Larcher, Ledwith, Lentz, McCoy, McCue, McCue, McCue, McCue, McCue, McCue, Naylor, Naylor, Quiroz-Rothe, Ribeiro, Schwarz, Sierra, Soliman, Stanley, Stanley, Valberg, Valberg, Valberg, Valberg, Valberg, Valentine, Valentine, Valentine, Valentine   +45 more
core   +2 more sources

Glycogen storage disease type I

, 2018
Shofiq Al-Islam, Ciléin Kearns, Daniel Bell   +2 more
openaire   +1 more source

Glycogen Storage Disease Type I and Bone: Clinical and Cellular Characterization. [PDF]

Calcif Tissue Int
Vai S, Falchetti A, Corbetta S, Bianchi ML, Alberio C, Carrara S, Gasperini S, Pretese R, Parisi L, Teti A, Maurizi A.   +10 more
europepmc   +1 more source

Safety and Efficacy of DTX401, an AAV8-Mediated Liver-Directed Gene Therapy, in Adults With Glycogen Storage Disease Type I a (GSDIa). [PDF]

J Inherit Metab Dis
Weinstein DA, Derks TG, Rodriguez-Buritica DF, Ahmad A, Couce ML, Mitchell JJ, Riba-Wolman R, Mount M, Sallago JB, Ross KM, van der Klauw MM, de Boer F, van der Schaaf C, Saavedra H, Martínez-Olmos M, Atanga E, Hosseini A, Mitragotri D, Crombez E.   +18 more
europepmc   +1 more source