Disease Information for Forbes disease (Cori disease, III) (Clinical Manifestations)

Amylo 1,6 glucosidase defici, amylo 1,6 glucosidase deficiency, CORI DIS, CORI DISEASE, CORI DISEASE TYPE III, CORIS DIS, Coris Disease, Cori's disease, DEBRANCHER DEFIC, Debrancher Deficiencies, debrancher deficiency, Debrancher deficiency glycogen storage disease, Debrancher enzyme deficiency, debrancher glycogen storage disease, DEFIC DEBRANCHER, Deficiencies Debrancher, Deficiency Debrancher, deficiency debrancher enzyme, Deficiency of 6 alpha D glucosidase, Deficiency of amylo 1,6 glucosidase, Deficiency of amylo 1,6 glucosidase (disorder), Deficiency of debranching enzyme, Deficiency of dextrin, Dextrinoses Limit, Dextrinosis Limit, Disease Cori, Disease Cori's, Disease Forbes, Forbes, FORBES DIS, FORBES DISEASE, Forbes' disease, GLYCOGEN DEBRANCHING ENZYME DEFIC, Glycogen Debranching Enzyme Deficiency, GLYCOGEN STORAGE DIS III, Glycogen storage disease III, GLYCOGEN STORAGE DISEASE TYPE III, Glycogen storage disease type III (disorder), GLYCOGENOSIS 03, Glycogenosis 3, glycogenosis type III, GSD III, Limit dextrin glycogen, Limit Dextrinoses, limit dextrinosis

Forbes Disease; Amylo-1,6-Glucosidase Deficiency; Cori Disease; Debrancher Deficiency; Glycogen Storage Disease III; Glycogenosis Type III; Limit Dextrinosis ;<br>

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Forbes disease (GSD-III) is one of several glycogen storage disorders (GSD) that are inherited as autosomal recessive traits; Symptoms are caused by a lack of the enzyme amylo-1,6 glucosidase (debrancher enzyme); This enzyme deficiency causes excess amounts of an abnormal glycogen to be deposited in the liver, muscles and, in some cases, the heart; There are two forms of this disorder. GSD-IIIA affects about 85% of patients with Forbes disease and involves both the liver and the muscles; GSD-IIIB affects only the liver;[NORD 2005]------------------------------------------------------

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