Inherited Myopathy of Great Danes

Other Names: Central core myopathy, IMGD
Affected Genes: BIN1
Inheritance: Autosomal Recessive
Mutation: Point Mutation
Breed(s): Great Dane

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Common Symptoms

Inherited Myopathy of Great Danes is a rare inherited degenerative muscle disorder. Affected dogs typically have an onset of symptoms before one year of age. Symptoms most commonly include difficulties exercising, muscle wasting and tremors brought on by exercise. Dogs may also have an abnormal posture with the hind legs tucked under the body and the tail extended. Occasionally dogs exhibit difficulty breathing. Most dogs that present with symptoms develop a severe, rapidly progressive form of the disease and are euthanized between 1 to 2 years of age. However, it has been reported that some dogs may have a more mild form of the disease and may live into adulthood without significant progression of the symptoms.


Testing Tips

Genetic testing of the BIN1 gene will reliably determine whether a dog is a genetic Carrier of inherited Myopathy of Great Danes. Inherited myopathy of Great Danes is inherited in an Autosomal Recessive manner in dogs meaning that they must receive two copies of the mutated gene (one from each parent) to develop the disease. In general, carrier dogs do not have features of the disease but when bred with another carrier of the same Mutation, there is a risk of having affected pups. Each pup that is born to this pairing has a 25% chance of inheriting the disease and a 50% chance of inheriting one copy and being a carrier of the BIN1 gene mutation. Reliable genetic testing is important for determining breeding practices. In order to eliminate this mutation from breeding lines and to avoid the potential of producing affected pups, breeding of known carriers to each other is not recommended. Dogs that are not carriers of the mutation have no increased risk of having affected pups.


There may be other causes of this condition in dogs and a normal result does not exclude a different mutation in this gene or any other gene that may result in a similar genetic disease or trait.


References

  • Böhm J, Vasli N, Maurer M, Cowling BS, Shelton GD, Kress W, Toussaint A, Prokic I, Schara U, Anderson TJ, Weis J, Tiret L, Laporte J. Altered splicing of the BIN1 muscle-specific exon in humans and dogs with highly progressive centronuclear myopathy. PLoS Genet. 2013 Jun;9(6). [PubMed: 23754947]
  • Feliu-Pascual Lujan A, Shelton GD, Targett MP, Long SN, Comerford EJ, McMillan C, Davies D, Rusbridge C, Mellor D, Chang KC, Anderson TJ. Inherited myopathy of great Danes. J Small Anim Pract. 2006 May;47(5):249-54. [PubMed: 16674719]
  • McMillan CJ, Taylor SM, Shelton GD. Inherited myopathy in a young Great Dane. Can Vet J. 2006 Sep;47(9):891-3. [PubMed: 17017655]