Dietary treatment in Glutaric Aciduria Type I

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Strauss et al. published an article describing the effects of a lysine-free, arginine-rich formula for the treatment  of Glutaric Aciduria Type I.  This successful treatment strategy takes advantage of the kinetics of the y+ blood brain barrier transporter, and presumably limits lysine transport into the CNS. They conclude that monitoring the ratio between lysine and arginine could be a successful strategy for treatment in GAI. This represents an exciting development in the treatment of a devastating disorder.  (Strauss et al., Mol Genet and Metab 104 (2011) 93-10)

Hilary Vernon, MD PhD

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