
Glycogen Storage Disease Type III - GeneReviews® - NCBI Bookshelf
Mar 9, 2010 · Glycogen storage disease type III (GSD III) is characterized by variable liver, cardiac muscle, and skeletal muscle involvement. GSD IIIa is the most common subtype, …
Glycogen storage disease type III - Wikipedia
Glycogen storage disease type III (GSD III) is an autosomal recessive metabolic disorder and inborn error of metabolism (specifically of carbohydrates) characterized by a deficiency in …
Glycogen storage disease type III: diagnosis, genotype, …
Glycogen storage disease type III (GSDIII) is a rare disorder of glycogenolysis due to AGL gene mutations, causing glycogen debranching enzyme deficiency and storage of limited dextrin. …
Biomarkers in Glycogen Storage Diseases: An Update - PMC
GSDI is diagnosed by identifying biallelic pathogenic variants: GSDIa, caused by a deficiency of glucose-6-phosphatase (G6Pase or G6PC1) catalytic activity; GSDIb, GSDIc and GSDId, …
Glycogen storage disease type III: diagnosis, genotype ... - PubMed
Glycogen storage disease type III (GSDIII) is a rare disorder of glycogenolysis due to AGL gene mutations, causing glycogen debranching enzyme deficiency and storage of limited dextrin. …
Glycogen storage disease type III - MedlinePlus
Glycogen storage disease type III (also known as GSDIII or Cori disease) is an inherited disorder caused by the buildup of a complex sugar called glycogen in the body's cells. The …
Glycogen storage disease type III (GSDIII) is a rare disorder of glycogenolysis due to AGL gene mutations, caus-ing glycogen debranching enzyme deficiency and storage of limited dextrin. …
Liver transplantation for glycogen storage disease types I, III, and IV
GSD type III is caused by a deficiency of glycogen debranching enzyme activity. The glycogen accumulated has a structure that resembles limit dextrin (glycogen with short outer chains). …
Challenges of Gene Therapy for the Treatment of Glycogen Storage ...
Glycogen storage diseases (GSDs) type I (GSDI) and type III (GSDIII), the most frequent hepatic GSDs, are due to defects in glycogen metabolism, mainly in the liver. In addition to …
Dietary lipids in glycogen storage disease type III: A systematic ...
Feb 16, 2020 · High fat diet may be beneficial in paediatric GSDIIIa patients with cardiac hypertrophy, but careful long-term monitoring for potential complications is warranted, such as …