The objective of this project is to define whether nutritional supplements (ornithine
alpha-ketoglutarate, glutamine, or citrate) capable of filling-up the citric acid cycle
(anaplerotic therapy) can improve hyperammonemia, glutamine levels, and outcome in patients
with propionic acidemia. Ornithine alpha-ketoglutarate, glutamine, and citrate are commonly
used as nutritional supplements specially by athletes to increase muscle strength. They can
be mixed with formula or other foods.
Phase:
Phase 1
Details
Lead Sponsor:
Nicola Longo
Collaborators:
National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) National Institutes of Health (NIH)