Hydroxychloroquine is available as the brand-name drug Plaquenil. Generic drugs usually cost less than the brand-name version. Hydroxychloroquine may be used as part of a combination therapy. Can plaquenil make you tired Plaquenil antimalarial drug Hydroxychloroquine forum Chloroquine CQ or hydroxychloroquine 4. The best charac-terized of these is the lysosomal storage disorder, Pompe disease, also known as glycogen storage disease type II. Pompe disease is caused by a mutation in the gene encoding acid a-glucosidase GAA, an enzyme that localizes to the lysosome, and hydrolyzes glycogen to glucose 5–7. Glycogen storage disease type 2, also known as Pompe disease or acid maltase deficiency disease, is an inherited metabolic disorder. While glycogen storage disease type 2 is a single disease, it may be classified in 2 forms according to the rates of disease progression, its severity and the age at which symptoms start. The classic infantile-onset starts before 12 month of age and involves the heart muscle myocardiopathy. Glycogen storage disease IIIa hypotonia, cardiomegaly muscle weakness, elevated creatine kinase CK, non-ketotic hypoglycemia* with more dramatic liver involvement than usually seen in Pompe disease. Autosomal recessive. Glycogen storage disease type IV hypotonia, cardiomegaly, muscle weakness, elevated CK. Hydroxychloroquine is used to treat lupus erythematosus and rheumatoid arthritis. It isn’t fully understood how this drug works to treat lupus erythematosus or rheumatoid arthritis. That means you may need to take it with other drugs. It treats malaria by killing the parasites that cause the disease. Pompe glycogen storage and hydroxychloroquine Glycogen storage disease type II - Wikipedia, Glycogen storage disease type 2 Genetic and Rare. Hydroxychloroquine in rheumatoid arthritis mechanism of actionQuinine in generic plaquenil What is glycogen and glycogen storage disease GSD? The body’s cells need a steady supply of fuel in order to function the right way. This fuel is a simple sugar called glucose. Glucose comes from breaking down the food we eat. The body uses as much glucose as it needs to function and stores the rest to use later. Glycogen Storage Disease GSD - Cleveland Clinic. Pompe Disease Glycogen storage disease, type II; Acid.. Pompe disease - Genetics Home Reference - NIH. Pompe disease, also known as acid maltase deficiency AMD or glycogen storage disease II, is a rare, inherited glycogen storage disease that affects the heart and skeletal muscles. There are two types of Pompe disease infantile-onset and noninfantile-onset juvenile or adult. Pompe is classified as a metabolic muscle Synonyms glycogen storage disease type II; acid maltase deficiency Pompe's disease is a glycogen storage disorder. Deficiency of the lysosomal enzyme alpha-1,4-glucosidase acid maltase leads to the accumulation of glycogen in many tissues A glycogen storage disease GSD, also glycogenosis and dextrinosis is a metabolic disorder caused by enzyme deficiencies affecting either glycogen synthesis, glycogen breakdown or glycolysis glucose breakdown, typically within muscles and/or liver cells. GSD has two classes of cause genetic and acquired.