Glycoscience - White, South Dakota

Currently lambs affected with GM1 gangliosidosis are not allowed into USDA inspected harvest channels. When GM1 lambs are harvested at 4-6 months of age, in order to maximize the yield of GM1, neurological signs may be observed which prohibits them from entering inspected meat slaughter channels. The harvest of the GM1 for pharmaceutical use has been calculated to be profitable after harvest of tissues. However, the remaining carcass could be consumed if it can be verified to have the composition of meat utilized in inspected channels.

GM1 ganglioside is potentially a breakthrough treatment for people who suffer from Huntingtonís disease, a rare but fatal genetic neurologic disorder. Glycoscience Research Inc. has developed a natural source for GM1 ganglioside from genetically selected lambs that are homozygous for a single base mutation in the beta-galactosidase gene. Affected lambs accumulate 40 x levels of GM1 ganglioside in their tissues. These lambs represent the only viable source to produce this life saving chemical. Researchers and Pharmaceutical companies have tried to synthesize GM1 for the past 25 years, yet these lambs, according to Dr. Steven Hersch of Massachusetts General Hospital, a collaborator on the ovine GM1 ganglioside for HD project, represent the best hope HD patients have for a useful treatment. Currently, lambs are harvested at approximately 5 months of age, but since they display neurologic symptoms associated with the massive accumulation of GM1 ganglioside, they are deemed unsuitable for inclusion in the food chain. While the value of the carcass is minimal compared to its pharmaceutical value, we believe that if the meat is shown to be safe and wholesome, and equal to normal lamb, there will be no reason for exclusion from the human food chain if all other criteria for inclusion are met. GM1 ganglioside is a normal glycolipid component of the cell membranes of all mammals, and the safety of the molecule has been demonstrated in thousands of human patients when produced from bovine brain. While the diversion of a few research lambs is of little consequence, we anticipate that roughly 100,000 lambs will needed yearly to treat HD, and when combined with the millions of lambs needed for Parkinsonís disease and Alzheimerís disease, would represent a substantial and unnecessary production loss that can be returned to cooperator producers as well as provide an additional protein supply for consumers. This study is intended to establish the baseline chemical and nutritional attributes of GM1 lamb compared to normal lamb.

The overall objective for this project is to provide a complete compositional analysis of meat product from GM1 gangliosidosis lambs compared to normal commercially available lamb.

Specific Aim 1: Analyze meat samples from GM1 lambs and commercial lambs for ash, moisture, protein, fat and carbohydrate content.

Specific Aim 2: Analyze GM1 lamb and normal commercially available lamb for ganglioside composition