Limb-Girdle Muscular Dystrophy (Dachshund Type)

Other Names: limb-girdle muscular dystrophy 2D, LGMD, LGMD2
Affected Genes: SGCA
Inheritance: Autosomal Recessive
Mutation: chr9:26117203 (canFam4): G/A
Breed(s): Dachshund, Miniature Longhaired Dachshund, Miniature Smooth Dachshund, Miniature Wirehaired Dachshund, Standard Longhaired Dachshund, Standard Smooth Dachshund, Standard Wirehaired Dachshund

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Common Symptoms

Limb-girdle Muscular Dystrophy (Dachshund Type) is an inherited neuro-muscular disease affecting dogs. Affected dogs typically present with clinical signs within 7 to 17 months of age. This form of muscular dystrophy presents with changes in muscular physiology that may only be detected during a veterinary examination of blood chemical markers. Some dogs with this disease have exercise intolerance, progressive weakness, and difficulty eating and drinking. Affected dogs can develop complications associated with aspiration pneumonia. Changes in the color of urine may result from the kidney filtering the products of abnormal muscle metabolism.

Testing Tips

Genetic testing of the SGCA gene in dogs will reliably determine whether it is a genetic Carrier of Limb-girdle muscular dystrophy (Dachshund Type). This disease is inherited in an Autosomal Recessive manner. This means that dogs 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 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 SGCA gene mutation. Reliable genetic testing is important for determining breeding practices. To eliminate this mutation from breeding lines and avoid the potential of producing affected pups, breeding of known carriers to each other is not recommended. Dogs that do not carry the mutation are not at increased risk of having affected pups. However, because there are multiple types of muscular dystrophy caused by mutations in other genes, a normal result in SGCA does not exclude muscular dystrophy in a pedigree.

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

  • Mickelson JR, Minor KM, Guo LT, Friedenberg SG, Cullen JN, Ciavarella A, Hambrook LE, Brenner KM, Helmond SE, Marks SL, Shelton GD. Sarcoglycan A mutation in miniature dachshund dogs causes limb-girdle muscular dystrophy 2D. Skelet Muscle. 2021 Jan 7;11(1):2. [PubMed: 33407862]