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L-2-HGA in Stafordshire BullTerriers
L-2-hga(L-2-hydroxglutaric aciduria) in Staffordshire Bull Terriers is a neurometabolic disorder characterized by elevated levels of L-2-hydroxyglutaric acid in urine,plasma and cerebrospinal fluid.
L-2-HGA affects the central nervous system.with clinical signs usually apparent between 6 months and one year.Although it can appear later on.Symptoms include epileptic seizures,wobblt gait,tremors,muscle stiffness as a result of exercise or excitement and altered behaviour.
The mutation or change to the structure of the gene,probably occurred spontaneously in a single dog but once in the population has been inherited from generation to generation like any other gene.The disorder shows an autosomal recessive mode of inherintence:two copies of the defective gene,one from each parent,have to be present for a dog to be affected by the disease.Individuals with only one copy of the defective gene are carriers,and show no syptoms but can pass the defective gene to their offspring.When two healthy carriers are bred,25% of the offspring will be affected,25% will be clear,and the remaining 50% will be carriers.
The mutation responsible for the disease has recently been identified at the Animal Health Trust.Using the information from this research,we have developed a DNA test for the disease.This test not only diagnoses dogs affected with the disease but can also detect affected puppies.Carriers could not be detected by the tests previously available,which involved resonance imaging.Under most circumstances there will be a much greater number of carriers than affected animals in a population since they represent a hidden reservoir of the disease that can produce affected dogs at any time.The test is available now and information on submitting samples follows.Breeders will be sent results identifying their dog as belonging to one of three categories.
CLEAR: The dog has two copies of the normal gene and will neither develop L-2-HGA,nor pass a copy of the gene to any of it's offspring.
CARRIER-the dog has one copy of the normal gene and one copy of the mutant gene that causes L-2-HGA.It will not develop L-2-HGA but will pass on the gene for the disease to 50% of it's offspring.
AFFECTED:the dog has two copies of the defective gene and is affected with L-2-HGA.It will develop the disease sometime in it's lifetime.
Carriers can still be bred to clear dogs.On average,50% of such a litter will be clear and 50% will be carriers.There will be no affected's from this breeding.DNA tests can be used to confirm whether or not they are clear or carriers.
Information on how to test for this disease and Hereditary Cateracts is available through the websites at the top of the page.
