Glycogen storage disease type III

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Glycogen storage disease type III

Metabolism

Metabolism

Citric acid cycle

Electron transport chain and oxidative phosphorylation

Gluconeogenesis

Glycogen metabolism

Pentose phosphate pathway

Physiological changes during exercise

Amino acid metabolism

Nitrogen and urea cycle

Fatty acid synthesis

Fatty acid oxidation

Ketone body metabolism

Cholesterol metabolism

Essential fructosuria

Hereditary fructose intolerance

Pyruvate dehydrogenase deficiency

Glucose-6-phosphate dehydrogenase (G6PD) deficiency

Lactose intolerance

Glycogen storage disease type II (NORD)

Glycogen storage disease type III

Glycogen storage disease type IV

Glycogen storage disease type V

Mucopolysaccharide storage disease type 1 (Hurler syndrome) (NORD)

Mucopolysaccharide storage disease type 2 (Hunter syndrome) (NORD)

Hartnup disease

Ornithine transcarbamylase deficiency

Phenylketonuria (NORD)

Cystinuria (NORD)

Maple syrup urine disease

Abetalipoproteinemia

Familial hypercholesterolemia

Hypertriglyceridemia

Disorders of carbohydrate metabolism: Pathology review

Disorders of amino acid metabolism: Pathology review

Disorders of fatty acid metabolism: Pathology review

Dyslipidemias: Pathology review

Glycogen storage disorders: Pathology review

Lysosomal storage disorders: Pathology review

Glycogen storage disease type I

Carbohydrates and sugars

Fats and lipids

Folate (Vitamin B9) deficiency

Vitamin B12 deficiency

Fat-soluble vitamin deficiency and toxicity: Pathology review

Zinc deficiency and protein-energy malnutrition: Pathology review

Water-soluble vitamin deficiency and toxicity: B1-B7: Pathology review

Nucleotide metabolism

Purine and pyrimidine synthesis and metabolism disorders: Pathology review

Gout and pseudogout: Pathology review

Acute intermittent porphyria

Key Takeaways

Glycogen storage disease type III is one of the genetic disorders affecting how the body stores and uses glycogen. People with glycogen storage disease type III have a deficiency of the glycogen debrancher enzyme (α-1,6-glucosidase), one of the enzymes that help to break down glycogen. The symptoms of glycogen storage disease type III generally appear during childhood and include generalized muscle weakness, hepatosplenomegaly, fasting hypoglycemia, and ketosis.