Glycogen storage disease 160851 211736211 2008-05-11T21:38:46Z Aunt Entropy 6417583 /* Types */ {{Infobox_Disease | Name = {{PAGENAME}} | Image = Glycogen.png | Caption = [[Glycogen]] | DiseasesDB = | ICD10 = {{ICD10|E|74|0|e|70}} | ICD9 = {{ICD9|271.0}} | ICDO = | OMIM = | MedlinePlus = | eMedicineSubj = | eMedicineTopic = | MeshID = D006008 | }} '''Glycogen storage disease''' (synonyms: '''glycogenosis''', '''dextrinosis''') is any one of several [[inborn error of metabolism|inborn errors of metabolism]] that result from [[enzyme]] defects that affect the processing of [[glycogen]] synthesis or breakdown within [[muscle]]s, [[liver]], and other cell types. ==Types== There are nine diseases that are commonly considered to be glycogen storage diseases. (Although [[glycogen synthase]] deficiency does not result in storage of extra glycogen in the liver, it is often classified with the GSDs as type 0 because it is another defect of glycogen storage and can cause similar problems.) {| class="wikitable" | '''Number''' || '''Enzyme deficiency''' || '''Eponym''' |- | [[Glycogen storage disease type I|GSD type I]] || [[glucose-6-phosphatase]] || [[von Gierke's disease]] |- | [[Glycogen storage disease type II|GSD type II]] || [[acid maltase]] || [[Pompe's disease]] |- | [[Glycogen storage disease type III|GSD type III]] || [[glycogen debrancher]] || [[Cori's disease]] or [[Forbe's disease]] |- | [[Glycogen storage disease type IV|GSD type IV]] || [[glycogen branching enzyme]] || [[Andersen disease]] |- | [[Glycogen storage disease type V|GSD type V]] || [[muscle glycogen phosphorylase]] || [[McArdle disease]] |- | [[Glycogen storage disease type VI|GSD type VI]] || [[liver glycogen phosphorylase]] || [[Hers' disease]] |- | [[Glycogen storage disease type VII|GSD type VII]] || [[muscle phosphofructokinase]] || [[Tarui's disease]] |- | [[Glycogen storage disease type IX|GSD type IX]] || [[phosphorylase kinase]] || - |- | [[Glycogen storage disease type XI|GSD type XI]] || [[glucose transporter]] || [[Fanconi-Bickel syndrome]] |- | [[Glycogen storage disease type 0|GSD type 0]] || [[glycogen synthase]] || - |} {{Carbohydrate metabolic pathology}} [[Category:Inborn errors of metabolism]] [[Category:Hepatology]] [[de:Glykogenspeicherkrankheit]] [[es:Glucogenosis]] [[fr:Maladie génétique du métabolisme des glucides]] [[it:Glicogenosi]] [[ja:糖原病]] [[pl:Choroby spichrzeniowe glikogenu]] [[pt:Glicogenose]] [[ru:Гликогенозы]]