Glycogen Storage Disease Type IA, Von Gierke Disease, GSD IA


Glycogen is the form in which our bodies store sugar. It is a long, complex molecule that is primarily stored in the liver, heart, kidney, and muscle. Glycogen is those tissues’ primary energy source. In GSD, glycogen accumulates to abnormal levels, leading to enlargement and malfunction of these vital organs. Dogs with Type I GSD have very severe symptoms and are markedly hypoglycemic.

  • Signs and symptoms

    Puppies with this disease often present severely hypoglycemic: lethargic, weak, and possibly seizing.

    Signs typically develop after only a few weeks, and most puppies with this disease die before two months of age.

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  • Diagnosis

    Genetic and laboratory testing are used to diagnose this disease.

  • Treatment

    There is no treatment except supportive care for the clinical signs the affected dog develops.

  • What to do if your dog is at risk


    • Focus on palliative therapies to increase your dog's comfort and quality of life.

  • Genetic Information

    This variant was first described in the German Pinscher.

    This variant is inherited in an autosomal recessive manner, meaning that a dog requires two copies of the variant to show signs of Glycogen Storage Disease Type IA (GSDIA).

    Gene names:

    G6PC1 Exon 5 ‐ chr

    Inheritance type:


  • Breeds affected

    This health condition affects the following breeds

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