Glycogen storage disease type Ia (GSDIa; OMIM#232200), also known as von Gierke disease, is an inborn error of carbohydrate metabolism caused by bi-allelic pathogenic variants in the …

Dec 23, 2019 · Glycogen storage diseases are a group of disorders in which stored glycogen cannot be metabolized into glucose to supply energy and to maintain steady blood glucose …

Glycogen storage disease type I (also known as GSDI or von Gierke disease) is an inherited disorder caused by the buildup of a complex sugar called glycogen in the body's cells. Explore …

Apr 19, 2006 · Glycogen storage disease type I (GSD I) is characterized by accumulation of glycogen and fat in the liver and kidneys resulting in hepatomegaly and nephromegaly. …

Glycogen storage disease type I (GSD I) is an inherited disease that prevents the liver from properly breaking down stored glycogen, which is necessary to maintain adequate blood sugar …

Glycogen storage disease due to glucose-6-phosphatase deficiency type Ia is an inherited disorder caused by the buildup of a complex sugar called glycogen in the body's cells. The …

Aug 8, 2023 · Glycogen storage disease type I (GSD I), also known as Von Gierke disease, is an inherited disorder caused by deficiencies of specific enzymes in the glycogen metabolism …

Nov 6, 2014 · Glycogen storage disease type I (GSD I) is a rare disease of variable clinical severity that primarily affects the liver and kidney.

Sep 1, 2017 · Glycogen storage disease type Ia (GSD-Ia) is an autosomal recessive metabolic disorder caused by a deficiency in glucose-6-phosphatase-α (G6Pase-α or G6PC) that is …

Glycogen storage disease type Ia (GSD-Ia) is an autosomal recessive metabolic disorder caused by a deficiency in glucose-6-phosphatase-α (G6Pase-α or G6PC) that is expressed primarily in …