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


Progress report on Current National Sheep Industry Improvement Program:


Title:  Comparison of meat composition of lambs affected with GM1 gangliosidosis to retail lamb


Investigators: S. Holler, L. Holler, K. Underwood, T. Hoffman



Progress Report:

The SDSU Meat Chemistry Lab has completed analysis of moisture, fat, protein, and ash of the 20 retail lamb legs and the moisture, fat, and ash content of the 20 GM1 gangliosidosis lamb legs. The 20 GM1 will be reanalyzed for protein content in the next month as our protein analysis equipment failed and erased the data for these samples from the previous analysis. This analysis is expected to be complete by the beginning of March. Upon completion of composition Keith Underwood and Travis Hoffman will begin analysis of the data to determine any differences in composition between the retail lamb and GM1 gangliosidosis affected lamb legs.

Progress Report for Dr. Jon Mitchell:

Briefly, we have routinely isolated GM1 using Larry's protocol from his patent application and neither afflicted nor unafflicted muscle appears to have very much GM1, and both muscle types seem to have the comparative amounts.  We have attempted a few experiments using a fluorescent antibody which binds to GM1 (from Invitrogen).  Both samples appear to exhibit the same amounts of fluorescence and they are in tiny amounts also.  We are using a purified GM1 control sample to monitor techniques that we purchased from Avanti.  We have not had a chance to quantify this though.  We are planning to get to this within the next two weeks.  We did try some nuclear magnetic resonance (NMR) analysis as we got a new one hooked up early December, but we did not have near the amount of GM1 to detect.  Disappointing that the NMR needs mgs of GM1.  We will probably make a trip to South Dakota State University to use their mass spectrophotometry (MS) as ours is misbehaving right now.  


*Dr. Jon Mitchell, Assistant Professor of Biology at Northern State University, Aberdeen, SD joined the project this summer. Dr. Underwood provided him with samples from both commodity and GM1 legs of lamb. Jon and his students are working on the GM1 ganglioside analysis.