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Intracellular P and ATP in X-Linked Hypophosphatemia

Program Faculty & Summary

Gerald Shulman, MD, PhD

Gerald Shulman is a well-known clinical investigator in the field of carbohydrate metabolism and diabetes. His study examined the role of intracellular phosphate, as pertains to energy metabolism. Dr. Shulman notes: “In preliminary studies we have found that dietary Pi-deprivation in normal mice results in severe hypophosphatemia and a 50% reduction in skeletal muscle ATP synthesis as measured by in vivo 31P-MRS saturation transfer methods. In addition, these mice exhibit reduced whole body energy expenditure, activity, and oxygen consumption. We examined intracellular phosphate metabolism, mitochondrial ATP synthesis and whole body energy metabolism in hypophosphatemic and euphosphatemic Hyp mice.

These studies will provide important new insights into the role of intracellular phosphate metabolism on the regulation of mitochondrial and whole body energy metabolism under normal physiological conditions as well as provide new insights into intracellular phosphate metabolism in patients with X-Linked Hypophosphatemia.”

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Representative spectra of a 31P saturation transfer performed in skeletal muscle.