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Restricted ketogenic diet enhances the therapeutic action of N‐butyldeoxynojirimycin towards brain GM2 accumulation in adult Sandhoff disease mice
Authors:Christine A Denny  Karie A Heinecke  Youngho P Kim  Rena C Baek  Katrina S Loh  Terry D Butters  Roderick T Bronson  Frances M Platt  Thomas N Seyfried
Institution:1. Department of Biology, Boston College, Chestnut Hill, Massachusetts, USA;2. Present address: Department of Biological Sciences, Columbia University, New York, New York, USA;3. Present address: Signal Transduction Program, The Burnham Institute for Medical Research, La Jolla, California, USA;4. Present address: Division of Pulmonary, Allergy, and Critical Care Medicine, Columbia University College of Physicians and Surgeons, New York, New York, USA;5. The Glycobiology Institute, Department of Biochemistry, University of Oxford, South Parks Road, Oxford, UK;6. The Dana Farber/Harvard Comprehensive Cancer Center, Boston, Massachusetts, USA;7. Department of Pharmacology, University of Oxford, Mansfield Road, Oxford, UK
Abstract:
Keywords:GA2  ganglioside  GM2  ketogenic diet  lysosomal storage disease  Sandhoff disease
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