succinic semialdehydeの例文

• Finally, succinic semialdehyde dehydrogenase levels can be measured in cultured leukocytes of the patient.
• The succinic semialdehyde can be converted into either succinic acid by SSADH or to GHB by the enzyme succinic semialdehyde reductase.
• The succinic semialdehyde can be converted into either succinic acid by SSADH or to GHB by the enzyme succinic semialdehyde reductase.
• GABA is metabolized by successive transamination and oxidation to yield succinic semialdehyde and succinic acid respectively via the catalyzing effects of GABA transaminase.
• It is important in the degradation of ?-aminobutyric acid in humans, and deficiency of the enzyme causes serious health effects ( succinic semialdehyde dehydrogenase deficiency ).
• Succinic Semialdehyde Dehydrogenase Deficiency is an autosomal-recessive gene disorder where mutations in the ALDH5A1 gene results in the accumulation of gamma-Hydroxybutyric acid ( GHB ) in the body.
• Vigabatrin is also used to treat seizures in succinic semialdehyde dehydrogenase deficiency ( SSADHD ), which is an inborn GABA metabolism defect that causes intellectual disability, hypotonia, seizures, GHB ).
• It has been proposed as a treatment for GHB overdose in humans as well as the genetic metabolic disorder succinic semialdehyde dehydrogenase deficiency ( SSADHD ), but has never been developed for clinical use.
• 4-Aminobutyrate aminotransferase ( ABAT ) is responsible for catabolism of gamma-aminobutyric acid ( GABA ), an important, mostly inhibitory neurotransmitter in the central nervous system, into succinic semialdehyde.
• Both of the metabolic breakdown pathways shown for GHB can run in either direction, depending on the concentrations of the substances involved, so the body can make its own GHB either from GABA or from succinic semialdehyde.
• However, because of the deficiency, the final intermediate of the GABA degradation pathway, succinic semialdehyde, accumulates and cannot be oxidized to succinic acid and is therefore reduced to gamma-hydroxybutyric acid ( GHB ) by gamma-hydroxybutyric dehydrogenase.