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Glycogen Storage Disease Type IV (Andersen's Disease)

An autosomal recessive metabolic disorder due to a deficiency in expression of glycogen branching enzyme 1 (alpha-1,4-glucan-6-alpha-glucosyltransferase), resulting in an accumulation of abnormal GLYCOGEN with long outer branches. Clinical features are MUSCLE HYPOTONIA and CIRRHOSIS. Death from liver disease usually occurs before age 2.
Also Known As:
Andersen's Disease; Glycogenosis, Type IV; Amylopectinosis; Andersen Disease; Deficiency, Brancher; Gbe1 Deficiency; Glycogen Branching Enzyme Deficiency; Glycogen Storage Disease Type 4; Glycogenosis IV; Type IV Glycogenosis; Amylopectinoses; Andersens Disease; Brancher Deficiencies; Deficiencies, Brancher; Deficiencies, Gbe1; Deficiency, Gbe1; Disease, Andersen; Disease, Andersen's; Gbe1 Deficiencies; Glycogenoses, Type IV; Glycogenosis 4s; Glycogenosis IVs; Type IV Glycogenoses; Brancher Deficiency; Glycogenosis 4
Networked: 117 relevant articles (0 outcomes, 7 trials/studies)

Disease Context: Research Results

Related Diseases

1. Glycogen Storage Disease Type I
2. Glycogen Storage Disease (Glycogenosis)
3. Fibrosis (Cirrhosis)
4. Glycogen Storage Disease Type III (Cori's Disease)
5. Fucosidosis

Experts

1. Chen, Yuan-Tsong: 5 articles (02/2012 - 04/2006)
2. Minassian, Berge A: 4 articles (01/2022 - 09/2007)
3. Akman, H Orhan: 4 articles (11/2020 - 10/2006)
4. Bali, Deeksha S: 4 articles (06/2012 - 03/2008)
5. DiMauro, S: 4 articles (12/2010 - 06/2001)
6. Kishnani, Priya S: 3 articles (01/2017 - 01/2016)
7. Sun, Baodong: 3 articles (01/2017 - 01/2016)
8. Yi, Haiqing: 3 articles (01/2017 - 01/2016)
9. Craigen, William J: 3 articles (12/2015 - 11/2011)
10. DiMauro, Salvatore: 3 articles (12/2015 - 04/2004)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Glycogen Storage Disease Type IV:
1. EnzymesIBA
2. GlycogenIBA
3. chitotriosidaseIBA
4. Proteins (Proteins, Gene)FDA Link
5. 1,4-alpha-Glucan Branching Enzyme (Glycogen Branching Enzyme)IBA
6. polyglucosanIBA
7. AmylopectinIBA
8. Polysaccharides (Glycans)IBA
9. Fibrinogen (Factor I)FDA Link
10. Cyanamide (Calcium Carbimide)IBA

Therapies and Procedures

1. Liver Transplantation
2. Therapeutics
3. Heart Transplantation (Grafting, Heart)
4. Transplantation