PPT-Glycogen Storage Diseases:

Objectives Structure of Glycogen Anabolism Vs Catabolism mechanisms Glycogen storage disorders Overview of Glycogen Metabolism Excess glucose stored as glycogen Glucose units joined by α. DR AMINA TARIQ. BIOCHEMISTRY. Polysaccharides. 2 types: . HOMO polysaccharides. (all 1 type of monomer) . glycogen, starch, cellulose, chitin . HETERO polysaccharides . (different types of monomers). Maltose, lactose, . trehalose. , sucrose. Fructose, mannose, . galactose. Feeder pathways for . glycolysis. 2. Catabolism of cellular glycogen. Glycogen phosphorylase catalyzes an attack by Pi on the (. C483 Spring 2013. 1. Why . does glycolysis produce more energy (more ATP) from glucose units released by glycogen degradation than from free glucose?. A) Limit dextrin contains additional molecules.. By controlling their concentration. Control of synthesis (activation or repression). Degradation. By controlling the availability of substrate. Production, degradation, . compartmentation. of substrate. MLAB 2401: Clinical Chemistry. Keri Brophy-Martinez. Introduction. 2. Organisms rely on the oxidation of complex organic compounds to obtain energy. Three general types of compounds provide chemical energy to our cells. By controlling their concentration. Control of synthesis (activation or repression). Degradation. By controlling the availability of substrate. Production, degradation, . compartmentation. of substrate. Regulation of . Glycogenolysis. Figure modified from . Yan, A., et al (2016) In J . Biol. . Sci. 12(12):1544-1554. and . Servier. Medical Art. GDP. cAMP. Protein Kinase A. (PKA). PKA. Regulation of . consists of 34 exons and widely expresses in liver, muscle, kidney, and lymphoblastoid cells []. GDE is a 175-kD monomeric protein containing two independent catalytic domains, amylo-1-6-glucosidase ( ...CARBOHYDRATE-LOADING:A SAFE AND EFFECTIVE METHOD OFIMPROVING ENDURANCE PERFORMANCERichard T. Beeker, M.A.Ed.Richard G. Israel, Ed.D.Richard T. Beeker is an exercise physiologist with Structure Hous 1 Metabolism of Carbohydrates Diseases Associated with Glycogen Degradation Paper : 04 Metabolism of carbohydrates Module : 28 Diseases Associated with Glycogen Degradation Dr. Ramesh Kothari, 2018) Vol. 70 (9), Page 1 539 - 1543 1539 Received: 16 /12/2017 DOI: 10.12816/0044680 Accepted: 26 /12/2017 Glycogen Storage Disease in Pediatric Population Yara Mofarih Assiri 1 , Marium Mohammed . Suud. . khalifa. Diseases caused by mutation in structural proteins:. Marfan"s. syndrome:. . autosomal dominant disease of connective tissue (. fibrillin. ), both quantitative &qualitative defects have been noted.. Lippincot’s. . Ilustrated. reviews: Biochemistry. Glucose Synthesis is Required for Survival. Brain is dependent on glucose 120g/day. Body glucose reserve is limited. ≈ 20 g (extra cellular fluid). Glycogen storage diseases (GSDs) occur when a defective enzyme is involved in a pathway for glycogen storage or degradation.. A micrograph shows an excess of stored glycogen (blue) in a liver biopsy of a patient with Cori’s disease..