Defective succinic semialdehyde dehydrogenase activity in 4-hydroxybutyric aciduria
β Scribed by K. M. Gibson; L. Sweetman; W. L. Nyhan; G. Lenon; P. Divry
- Book ID
- 104797008
- Publisher
- Springer
- Year
- 1984
- Tongue
- English
- Weight
- 308 KB
- Volume
- 142
- Category
- Article
- ISSN
- 0340-6997
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π SIMILAR VOLUMES
Escherichia coli mutants, unable to grow on 4hydroxyphenylacetate, have been isolated and found to be defective in the NAD-dependent succinate semialdehyde dehydrogenase. When the mutants are grown with 4-aminobutyrate as sole nitrogen source an NAD-dependent succinate semialdehyde dehydrogenase see
## Abstract We report the successful treatment using lowβdose vigabatrin (21.5β34mg/kg/day) of a 10βyearβold girl with succinic semialdehyde dehydrogenase (SSADH) deficiency We verified that 4βhydroxybutyric acid (GHB) concentrations in serum, cerebrospinal fluid, and urine continuously decreased i