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New insight into childhood metabolic disease

Glutaric acidemia type I is an inherited disorder similar to Huntington disease. In most, but not all, affected children, a period of normal development is followed by an irreversible brain injury triggered by a nonspecific illness. New research using a mouse model of GA-I has provided insight into the mechanisms underlying injury and age-dependent susceptibility to the disease and suggested a way to monitor children with the disease.

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Keywords: insight, childhood, metabolic, disease

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1. JCI table of contents: Oct. 11, 2007
   10-11-2007 · EurekAlert!
   This release contains summaries, links to PDFs and contact information for the following newsworthy papers to be published online, October 11, 2007, in the JCI, including: New insight into childhood metabolic disease; Mast cells get straight As; CD8+ T cells LAG behind; It’s good to talk: PGC-1-alpha helps skeletal muscle and pancreatic islets communicate; HRI controls anemia of inflammation with an iron fist; and others.
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