Despite its 19th century reputation as a ferocious and fearless competitor in the cruel sport of dog fighting, the modern, well-bred Staffordshire bull terrier (SBT) is an affectionate, friendly, and loyal companion. At only 24 to 38 pounds, the SBT’s impressive, muscular frame is now a relic from a distant time when dogs slept in the backyard instead of the bedroom and often proved their worth through the use of their agility, strength, tenacity, and teeth. Through nearly a century of careful selective breeding for temperament, the SBT has become as suitable for the family as they once were for the fighting ring. As with other purebred dogs, along the path of breed improvement, the SBT has developed some inherited diseases that have caused problems for SBT owners and breeders alike. One of the most concerning inherited diseases in the SBT is the neurometabolic disorder commonly referred to by the acronym L-2-HGA; short for L-2 hydroxyglutaric aciduria.
What is L-2-HGA?
Dogs affected with L-2-HGA lack functional copies of a protein important in eliminating L-2 hydroxyglutaric acid (a normal product of metabolism) from the body. As a result, L-2 hydroxyglutaric acid accumulates in the urine, blood, and cerebrospinal fluid. Though ...