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Glycogen Storage Disease Type III (Cori's Disease)

An autosomal recessive metabolic disorder due to deficient expression of amylo-1,6-glucosidase (one part of the glycogen debranching enzyme system). The clinical course of the disease is similar to that of glycogen storage disease type I, but milder. Massive hepatomegaly, which is present in young children, diminishes and occasionally disappears with age. Levels of glycogen with short outer branches are elevated in muscle, liver, and erythrocytes. Six subgroups have been identified, with subgroups Type IIIa and Type IIIb being the most prevalent.
Also Known As:
Cori's Disease; Forbes Disease; Amylo-1,6-Glucosidase Deficiency; Cori Disease; Deficiency, Debrancher; Glycogen Debrancher Deficiency; Glycogen Storage Disease III; Glycogen Storage Disease Type 3; Amylo 1,6 Glucosidase Deficiency; Amylo-1,6-Glucosidase Deficiencies; Coris Disease; Debrancher Deficiencies; Debrancher Deficiencies, Glycogen; Debrancher Deficiency, Glycogen; Deficiencies, Amylo-1,6-Glucosidase; Deficiencies, Debrancher; Deficiencies, Glycogen Debrancher; Deficiency, Amylo-1,6-Glucosidase; Deficiency, Glycogen Debrancher; Dextrinoses, Limit; Dextrinosis, Limit; Disease, Cori; Disease, Cori's; Disease, Forbes; Glycogen Debrancher Deficiencies; Glycogenosis 3s; Limit Dextrinoses; Debrancher Deficiency; Glycogen Debranching Enzyme Deficiency; Glycogenosis 3; Limit Dextrinosis
Networked: 135 relevant articles (1 outcomes, 7 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Glycogen Storage Disease (Glycogenosis)
2. Glycogen Storage Disease Type I
3. Glycogen Storage Disease Type IV (Andersen's Disease)
4. Liver Cell Adenoma (Hepatocellular Adenoma)
5. Fibrosis (Cirrhosis)

Experts

1. Kishnani, Priya S: 10 articles (12/2021 - 03/2007)
2. Okubo, Minoru: 7 articles (02/2015 - 01/2002)
3. Abdelhak, Sonia: 5 articles (09/2018 - 03/2012)
4. Tebib, Neji: 5 articles (09/2018 - 03/2012)
5. Murase, Toshio: 5 articles (09/2013 - 01/2002)
6. Comi, G P: 5 articles (03/2012 - 05/2002)
7. Lucchiari, Sabrina: 4 articles (10/2018 - 03/2012)
8. Pagliarani, Serena: 4 articles (10/2018 - 03/2012)
9. Labrune, Philippe: 4 articles (11/2017 - 12/2011)
10. Smit, G Peter A: 4 articles (01/2016 - 08/2007)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Glycogen Storage Disease Type III:
1. Glucose (Dextrose)FDA LinkGeneric
2. GlycogenIBA
3. amylo-1,6-glucosidaseIBA
4. Glycogen Debranching Enzyme System (Glycogen Debranching Enzyme)IBA
5. EnzymesIBA
6. LipidsIBA
7. DextrinsIBA
8. Proteins (Proteins, Gene)FDA Link
9. GlucosidasesIBA
10. Starch (Cornstarch)IBA

Therapies and Procedures

1. Liver Transplantation
2. High-Protein Diet
3. Enzyme Replacement Therapy
4. Ketogenic Diet
5. Therapeutics