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Limb-girdle Muscular Dystrophy, Type L3 (Discovered in the Miniature Dachshund)

Limb-girdle Muscular Dystrophy, Type L3 (LGMDR3) is a disorder that is characterized by progressive muscle wasting, particularly over the shoulders and hips, and changes in walking patterns. The associated genetic variant has been identified in the Miniature Dachshund

Found in

1 in 38,000 dogs

in our testing

Key Signs

Muscle wasting, Exercise intolerance, Stiff walking gait, Difficulty swallowing, Myoglobinuria

Age of Onset

0 to 2 yrs

Juvenile onset

Inheritance

Autosomal Recessive

For autosomal recessive disorders, dogs with two copies of the variant are at risk of developing the condition. Dogs with one copy of the variant are considered carriers and are usually not at risk of developing the disorder. However, carriers of some complex variants grouped in this category may be associated with a low risk of developing the disorder. Individuals with one or two copies may pass the disorder-associated variant to their puppies if bred.

Likelihood of the Condition

High likelihood

At risk dogs are highly likely to show signs of this disease in their lifetime.

What to Do

Here’s how to care for a dog with LGMDR3

Partner with your veterinarian to make a plan regarding your dog’s well-being, including any insights provided through genetic testing. If your pet is at risk or is showing signs of this disorder, then the first step is to speak with your veterinarian.

For Veterinarians

Here’s what a vet needs to know about LGMDR3

Limb-girdle muscular dystrophies are a group of inherited disorders which lead to progressive muscle dysfunction. The group is characterized by muscle weakness which, in dogs, has a tendency to affect muscles of the shoulders and hips. This variant in the SGCD gene, first discovered in the Miniature Dachshund, is associated with Limb-girdle Muscular Dystrophy, Type L3 (LGMDR3). Affected dogs typically start to show clinical signs between 7 to 17 months of age. Signs may vary in severity from subclinical myopathy to generalized weakness, exercise intolerance, stiff gait, dysphagia (difficulties swallowing) and myoglobinuria. Persistently elevated serum creatinine kinase (CK) activity is characteristic for this form of LGMD. Muscle biopsies in affected dogs can reveal changes consistent with muscular dystrophy.

As there is no cure for this disorder, therapy is limited to symptomatic treatments and general supportive care. Due to the progressive nature of LGMD, the average long-term prognosis is considered poor for affected dogs.

For Breeders

Planning to breed a dog with this genetic variant?

There are many responsibilities to consider when breeding dogs. Regardless of test results it is important that your dog is in good general health and that you are in a position to care for the puppies if new responsible owners are not found. For first time or novice breeders, advice can be found at most kennel club websites.

This disorder is autosomal recessive, meaning two copies of the variant are needed for a dog to be at an elevated risk for being diagnosed with the condition. A carrier dog with one copy of the Limb-girdle Muscular Dystrophy, Type L3 (Discovered in the Miniature Dachshund) variant can be safely bred with a clear dog with no copies of the Limb-girdle Muscular Dystrophy, Type L3 (Discovered in the Miniature Dachshund) variant. About half of the puppies will have one copy (carriers) and half will have no copies of the variant. Puppies in a litter which is expected to contain carriers should be tested prior to breeding. Carrier to carrier matings are not advised as the resulting litter may contain affected puppies. Please note: It is possible that disorder signs similar to the ones associated with this Limb-girdle Muscular Dystrophy variant could develop due to a different genetic or clinical cause.

Technical Details

Gene SGCA
Variant G>A
Chromosome 9
Coordinate 26,166,312

All coordinates reference CanFam3.1

References & Credit

Credit to our scientific colleagues:

Mickelson, J.R., Minor, K.M., Guo, L.T., Friedenberg, S.G., Cullen, J.N., Ciavarella, A., … Shelton, G.D. (2021). Sarcoglycan A mutation in miniature dachshund dogs causes limb-girdle muscular dystrophy 2D. Skelet Muscle, 11(1), 2. View the article