and the GABAB receptor early in the life of the Aldh5a1-/- mice can increase levels of GHB and enhance GABA release. Besides these effects, it has also been shown that "...a developmental down-regulation of GABAA receptor mediated neurotransmission in Aldh5a1-/- mice likely contributes to the progression of generalized convulsive seizures seen in mutant animals." Other studies have confirmed the relationship between elevated levels of GHB and MAP kinase in mutant animals contribute to profound myelin abnormalities. See also Inborn errors of metabolism References Further reading GeneReviews/NCBI/NIH/UW entry on Succinic Semialdehyde Dehydrogenase Deficiency NINDS - National Institute of Neurological Disorders and Stroke External links Amino acid metabolism disorders Autosomal recessive disorders Rare diseases Gamma-Hydroxybutyric acid