Glycogen Storage Disease Type II (GSDII)

Glycogen storage disease is sometimes known as Pompe’s disease or Glycogenesis, and is characterised by the deficiency or defective nature of the bodily enzymes that metabolises the body’s glycogen. Glycogen is a type of long term, slow release energy that is stored in the muscles and the liver, along with water. The muscles and liver then convert glycogen into glucose, which is in turn used to fuel the body.

When a dog suffers from glycogen storage disease, the enzymes requires for metabolising glycogen and turning it into glucose are missing or fail to function properly. This in turn leads to an overly high accumulation of glycogen in the body’s tissues, which causes them to become enlarged, causing dysfunction in major organs such as the heart, kidneys and liver.

Glycogen storage disease is relatively rare in dogs, but it is a hereditary condition which means that it can be passed on through the breed line from parent dogs to their offspring, and various different sub-types of the condition can be found in different breeds, depending on which gene mutation is behind them. While the condition is reasonably uncommon, it is fatal in the vast majority of affected dogs and so dogs from breed lines known to be affected by the condition should be tested prior to breeding.

In this article, we will look at the GSDII type of glycogen storage disease in dogs, including what type of dogs can be affected by the condition, how the condition is passed on from dog to dog, and how dogs can be tested for the condition. Read on to learn more.

More about glycogen storage disease Type II in dogs

Glycogen storage disease leads to the affected dog accumulating too much glycogen in their cells, which can lead to a dangerous enlargement of the major organs such as the heart, liver and kidneys. Glycogen storage disease comes in four different types, each of which work in slightly different ways and affect different combinations of dog breeds by means of heredity.

Type II glycogen storage disease, which is the form of the condition that we are looking at in this article, leads to a progressive and generalised muscle weakness in affected dogs, heart problems, and vomiting. The condition usually proves fatal in dogs, generally prior to the age of two.

What sort of dogs can be affected by it?

As a hereditary health condition, glycogen storage disease type II cannot be caught or transmitted between dogs other than by means of heredity, or inheriting the markers for the condition from the parent dogs.

This type of the condition is generally found in the Lapland dog breeds such as theFinnish Lapphund, Swedish Lapphund and Lapponian Herder, but if you want to find out if your own dog breed is at risk of the condition or check for other recognised hereditary health problems within their breed group, you can check The Kennel Club’s DNA screening scheme database.

How does the heredity of the condition work?

Glycogen storage disease type II is an autosomal recessive hereditary condition, which means that dogs must inherit a certain combination of faulty genes from each of their parents in order to develop the condition themselves. If only one of the parent dogs carries the condition and the other dog is clear, the offspring may become carriers for the condition but not suffer from it themselves, but they can then pass the condition on to their offspring, which is why it is so important to test for the condition in breeding stock to prevent it being passed on to their offspring.

If a dog inherits two copies of the gene fault, one from each parent, they will develop the condition themselves, while of course if neither parent dog is affected by or is a carrier for the condition, the puppies will not be either.

How can you test your dog for the condition?

In order to have your dog tested for the condition, you will need to ask your vet to take a DNA sample from your dog, which you then need to send off to one of the approved laboratories that can test the sample and return the results to you.

For a list of laboratories that can perform the test in the UK, check out this information on The Kennel Club’s website.

While glycogen storage disease type II is relatively rare in most breed lines, the importance of testing for the condition is often overlooked-however, the disease is ultimately fatal in affected dogs and cannot be prevented or cured, and so it is really important to ensure that potential breeding stock are tested for the condition first, in order to prevent it being passed on, and so, becoming more prevalent across the gene pool as a whole.