Mucopolysaccharidosis Type III in goats, commonly known as G6S (similar to human Mucopolysaccharidosis Type III, also known as Sanfilippo syndrome, Type D), is a metabolic disease caused by a genetic defect. It is a simple recessive genetic disorder. G6S was first identified in the Nubian breed of goat in the 1980s by researchers at the University of Michigan, USA. The symptoms in affected kids are similar to those observed in human children with Mucopolysaccharidoses. In addition to diagnosis through physical characteristics and biochemical tests (such as urinary metabolites of mucopolysaccharides), a molecular biotechnology method for detecting the G6S genotype was developed in 1995 by Leipprandt et al. at the University of Michigan. Many research teams have since worked on Mucopolysaccharidosis Type III therapy using goats as an animal model, with hopes that the findings may be applied to human Mucopolysaccharidosis therapy.

G6S is a serious genetic disease in Nubian goats. The goat industry in Taiwan is important, with the consumption of goat meat steadily growing. The main meat goat breeds in Taiwan are crossbreeds of Nubian and Taiwanese native goats. The researchers at the Taiwan Livestock Research Institute tests on 20 Nubian breeding goats (2 males and 18 females) collected from a goat farm in the southern region, based on the testing method developed by Leipprandt et al. The preliminary results showed that 25% (5/20) of the goats carried this defect gene. This confirmed the presence of the defect gene on the farm. The frequency of the defect gene in the existing Taiwanese goat population is high, not only in Nubian goats but possibly also in imported goats, many of which are graded crossbreeds. Six individuals with the G6S genotype were found to carry the defect gene, all of which were Nubian goats. To help the goat breeding industry easily identify and understand the G6S genotype, the following labels are used: AA for normal, AB for heterozygous, and BB for affected. The recommended breeding guidelines are: “AA with AA,” “AA with AB,” and “less AB with AB” for all goat farmers.

If a defect gene is present on the farm, it is recommended to test retained does and eliminate heterozygous does. If an exceptionally good heterozygous doe is selected for breeding, it is still possible to produce normal offspring. However, for those intending to breed goats, it is best to have all animals tested. Nowadays, goats sold worldwide (especially Nubian goats) are guaranteed to be free of defect genes across all generations. For further assistance, please contact the Genetics and Physiology Division at 06-5911211 ext. 2200.

The way to produce a defect is that both parents are carriers.

Do not use heterozygous bucks to spread the defect gene.

There is a chance for an excellent carrier buck to produce breeding candidate offspring.

Genetics and Physiology Division at 06-5911211 ext. 2200.