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SoftMaple GSDIIIa results
John C. Fyfe, D.V.M, Ph.D.
D.V.M., 1984, Washington-Oregon-Idaho Regional Program in Veterinary Medicine
Ph.D., 1994, University of Pennsylvania
Department of Microbiology and Molecular Genetics
5169 Biomedical Physical Sciences
Michigan State University
East Lansing, MI 48824
Glycogen storage disease type IIIa (GSDIIIa) is an inherited metabolic disorder that causes liver and skeletal muscle disease due to deficiency of the glycogen
debranching enzyme (GDE) and tissue storage of abnormally structured glycogen. This disorder was discovered in an extended family of curly coated retrievers
(CCR), with representatives from USA, Canada, and New Zealand, and is due to a single based deletion in the GDE gene. This disease, too, is an autosomal
recessive trait, and so the laboratory provides carrier testing for GSD IIIa in curly coated
A quick explanation is shown in the tables below. BB being a dog that does not have the disease and does not carry the recessive gene for GSDIIIa. Bb demonstrates a dog that does not have the disease, but does carry the recessive gene. bb represents a dog that is affected with the disease.
Dogs that are carriers need not be eliminated from breeding. Carriers bred to clear, non carriers, will produce some carriers and some non-carriers. These offspring may then be tested and used as valuable animals in a breeding program.
Think in terms of a stop light. Green means go... a non carrier dog, you can breed. Orange means caution... a dog that is a carrier, can be bred, but only to
non-carriers. Red means STOP! this is a dog with the disease. Even if it could be bred, all pups will be carriers.
produces all clear, not carrier offspring(BB)
The offspring af a breeding like this would all be clear of GSD based on the absence of the GSD mutation in both parents.
A non affected, not carrier dog (BB) A non affected carrier bitch (Bb).
produces all non affected pups, two of which would carry the GSDIIIa gene as a recessive trait (Bb)
No dogs would have the disease, but all offspring should be tested prior to breeding
one GSDIIIa affected pup(bb) and 2 pups that are carriers(Bb)
There is the potential for pups that will have Glycogen storage disease, and some will be carriers.
All pups are carriers, and should be only bred to non-carriers.
Some of the pups will have Glycogen storage disease, and some will be carriers.
These are just examples. I do not think anyone would breed two affected dogs.
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