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3-Hydroxyglutaric acid

In Glutaric Aciduria Type I (glutaryl-CoA dehydrogenase deficiency) elevated urinary levels of 3-hydroxyglutaric acid are found. Although glutaric acid excretion may be increased as well, this is not always the case. Furthermore, whereas increased urinary glutaric acid is also found in other diseases, elevated excretion of 3-hydroxyglutaric acid is specific for Glutaric Aciduria Type I.

- 3-hydroxyglutaric acid