Brain NAA is known to be elevated in Canavan’s disease but is usually decreased in association with loss of neuronal integrity. Elevated NAA is herein reported in murine models of sickle cell disease (SCD) and is associated with anemia, hypoxic and oxidative stress and may reflect increased glutathione turnover and / or increase demand for mitochondrial energy. These findings provide insight into why elevated NAA has been observed in children but not adults with SCD, and suggests a mechanism for increased susceptibility to stroke, and alternative roles for NAA in the brain.
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