Glycogen Storage Disease Type III (Cori's Disease)

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56 relevant articles (0 outcomes, 2 trials/studies) found for this Disease

Description: 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; Cori Disease; Deficiency, Debrancher; Coris Disease; Debrancher Deficiencies; Deficiencies, Debrancher; Dextrinoses, Limit; Dextrinosis, Limit; Disease, Cori; Disease, Cori's; Disease, Forbes; Limit Dextrinoses; Debrancher Deficiency; Glycogen Debranching Enzyme Deficiency; Glycogenosis 3; Limit Dextrinosis

Disease Context: Research Results

Congenital, Hereditary, and Neonatal Diseases and Abnormalities: 421

Inborn Genetic Diseases: 549

Inborn Errors Metabolism: 402

Inborn Errors Carbohydrate Metabolism

Glycogen Storage Disease: 920

Glycogen Storage Disease Type III: 56

Nutritional and Metabolic Diseases

Metabolic Diseases: 1267

Inborn Errors Metabolism: 402

Inborn Errors Carbohydrate Metabolism

Glycogen Storage Disease: 920

Glycogen Storage Disease Type III: 56

Related Diseases

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

Experts

1.	Gentry, Matthew S: 1 article (10/2007)
2.	Worby, Carolyn A: 1 article (10/2007)
3.	Saltiel, Alan R: 1 article (10/2007)
4.	Dixon, Jack E: 1 article (10/2007)
5.	Cheng, Alan: 1 article (10/2007)
6.	Zhang, Mei: 1 article (10/2007)
7.	Zhuang, Tai-feng: 1 article (02/2005)
8.	Qiu, Zheng-qing: 1 article (02/2005)
9.	Wei, Min: 1 article (02/2005)
10.	Huang, Shang-zhi: 1 article (02/2005)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Glycogen Storage Disease Type III:

1.	GlycogenIBA 10/01/2007 - "Taken together, these results indicate that binding to glycogen crucially regulates the stability of AGL and, further, that its ubiquitination may play an important role in the pathophysiology of both Lafora and Cori's disease." 10/01/2007 - "A role for AGL ubiquitination in the glycogen storage disorders of Lafora and Cori's disease." 02/01/2005 - "Mutation analysis of glycogen debrancher enzyme gene in five Chinese patients with glycogen storage disease type III]" 12/01/1990 - "We cultured fibroblasts from a skin biopsy from a patient with debrancher deficiency and examined the metabolism of glycogen in these cultured fibroblasts" 03/01/1981 - "Glycogen debrancher deficiency is reproduced in muscle culture." Order ALL the reference details at left...
2.	Glycogen Debranching Enzyme System (Glycogen Debranching Enzyme)IBA 01/01/2002 - "Glycogen storage disease type III (GSD III) is a rare autosomal recessive inherited disorder caused by a deficiency of the glycogen-debranching enzyme (AGL)" 07/31/2000 - "Glycogen storage disease type III (GSD III) is an autosomal recessive disorder caused by deficiency of glycogen-debranching enzyme (AGL)" 01/01/2000 - "Deficiency of glycogen debranching enzyme (AGL) activity causes glycogen storage disease type III (GSD-III)" 12/01/1999 - "Several different mutations in the glycogen-debranching enzyme gene AGL have been found in patients with glycogen storage disease type III (GSD III) to date, but no missense mutations have been reported for GSD III, only nonsense, splicing, and deletion/insertion lesions" 04/01/1998 - "Glycogen storage disease type III (GSD III) is an autosomal recessive disease caused by the deficiency of glycogen debranching enzyme (AGL)" Order ALL the reference details at left...
3.	amylo-1,6-glucosidaseIBA 06/01/2008 - "Glycogen storage disease type III (GSD-III) is an autosomal recessive disorder caused by the lack of amylo-1,6-glucosidase (AGL), one of the catalytic domains of the glycogen debranching enzyme" 06/01/2008 - "Glycogen storage disease type III (GSD III; OMIM 232400) is an autosomal recessive deficiency of the glycogen debrancher enzyme, amylo-1,6-glucosidase (EC 3.2.1.33)" 05/01/2002 - "Deficiency of amylo-1,6-glucosidase, 4-alpha-glucanotransferase enzyme (AGL or glycogen debrancher enzyme) is responsible for glycogen storage disease type III, a rare autosomal recessive disorder of glycogen metabolism" 02/01/1998 - "Glycogen storage disease type III, or Cori's disease, is caused by a deficiency of amylo-1,6-glucosidase (debranching enzyme), which leads to the storage of an abnormal glycogen in the liver and in skeletal and heart muscle" 03/01/1986 - "A 42-year-old man with adult-onset type III glycogenosis (Cori's disease) developed a gradually progressive polyneuropathy with markedly reduced activity of muscle amylo-1,6-glucosidase and glycogen accumulation within all elements of biopsied sural nerve, including axons, as shown by ultrastructural assessment." Order ALL the reference details at left...
4.	GlucosidasesIBA 11/30/1961 - "A case of "dextrinosis limit" (absence of amylo-1, 6-glucosidase)]" 04/01/1978 - "27 of them has type II glycogen disorders (Pompe's disease, with an intralysozymal deficit of acid maltase) and 6 with type III glycogen disorders Forbes disease, with a deficit in amylo-1-6-glucosidase)" 02/01/2000 - "The static glycogen storage myopathies include: GSD I or Pompe's disease (acid maltase or (-glucosidase deficiency), GSD II or Cori-Forbes disease (debranching enzyme deficiency), and GSD IV or Andersen's disease (branching enzyme deficiency)" Order ALL the reference details at left...
5.	Glucose (Dextrose)FDA LinkGeneric 01/01/1990 - "Changes in the isotopomer distribution of plasma 13C glucose were found only in glycogen storage disease type III patients and control subjects" 06/01/1968 - "Glucose and glycogen metabolism in erythrocytes from normal and glycogen storage disease type III subjects." 09/01/1989 - "Glucose carbon recycling, endogenous glucose production, and glucose turnover rates were measured, by stable isotope methodology, in five patients with glycogen storage disease type I (GSD-I), two patients with glycogen storage disease type III (GSD-III), and three control children" Order ALL the reference details at left...
6.	alpha-Glucosidases (Acid Maltase)IBA 07/01/1996 - "Deficiency of glycogen debranching enzyme and acid alpha-glucosidase cause glycogen storage disease type III and II, respectively" 04/01/1978 - "27 of them has type II glycogen disorders (Pompe's disease, with an intralysozymal deficit of acid maltase) and 6 with type III glycogen disorders Forbes disease, with a deficit in amylo-1-6-glucosidase)" 02/01/2000 - "The static glycogen storage myopathies include: GSD I or Pompe's disease (acid maltase or (-glucosidase deficiency), GSD II or Cori-Forbes disease (debranching enzyme deficiency), and GSD IV or Andersen's disease (branching enzyme deficiency)" Order ALL the reference details at left...
7.	Protein Isoforms (Isoforms)IBA 06/01/2006 - "Glycogenosis type III (Cori disease) is an autosomal recessive disorder caused by the deficiency of the glycogen debranching enzyme, encoded by the AGL gene, and existing in six isoforms alternately spliced in a tissue-specific way" Order ALL the reference details at left...
8.	Complementary DNA (cDNA)IBA 07/16/1996 - "Analysis of glycogen debranching enzyme (debrancher) cDNA from a patient with glycogen storage disease type III revealed a deletion of 124 base pairs" Order ALL the reference details at left...
9.	1,4-alpha-Glucan Branching Enzyme (Glycogen Branching Enzyme)IBA 02/01/2000 - "The static glycogen storage myopathies include: GSD I or Pompe's disease (acid maltase or (-glucosidase deficiency), GSD II or Cori-Forbes disease (debranching enzyme deficiency), and GSD IV or Andersen's disease (branching enzyme deficiency)" Order ALL the reference details at left...
10.	TransferasesIBA 06/01/1992 - "Assessments of debranching enzyme or debranching enzyme transferase activity in muscle can be used to predict whether patients with glycogen storage disease type III will develop myopathy and cardiomyopathy." Order ALL the reference details at left...

Therapies and Procedures

1.	Liver Transplantation 01/01/1994 - "The development of hepatocellular adenoma has been recognized in association with glycogen storage disease type I and, less often, with glycogen storage disease type III, but, to our knowledge, it has not been reported in glycogen storage disease type IV. We had the opportunity to study an 11-month-old male infant who underwent orthotopic liver transplantation for cirrhosis that developed in the setting of glycogen storage disease type IV. A clinically unsuspected hepatocellular adenoma was present in the explanted liver" Order ALL the reference details at left...
2.	Diet Therapy (Therapy, Diet) 07/01/1991 - "Diet therapy in severe clinical expression of debrancher deficiency." Order ALL the reference details at left...
3.	Cardiopulmonary Bypass (Heart-Lung Bypass) 09/01/2002 - "Perioperative management of a child with glycogen storage disease type III undergoing cardiopulmonary bypass and repair of an atrial septal defect." Order ALL the reference details at left...

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