NASDAQ: ALDX) presented novel data at the American Society of Human Genetics 2017 annual meeting demonstrating that ADX-102 reduced levels of gamma-hydroxybutyrate (GHB) and gamma-aminobutyrate (GABA) in a knockout mouse model of Succinic Semialdehyde
Dehydrogenase (SSADH) deficiency, the company said.
The presence of Catechol-1,2-dioxygenase (C12D) or Catechol-2,3-dioxygenase (C23D) was detected by the formation of products cis-cis muconic acid and 2-hydroxymuconic semialdehyde
(2-HMS) at 260 nm and 375 nm, respectively (Santos and Linardi, 2004).
elata, appeared to irreversibly inactivate the succinic semialdehyde
dehydrogenase (SSADH) of bovine brain in in vitro conditions (Table 3, entry 2; Fig.
Enzymatic Conversion of [beta]-Hydroxypropionate to Malonic Semialdehyde
A ketogenic diet rescues the murine succinic semialdehyde
dehydrogenase deficient phenotype.
Emma was diagnosed with the condition Succinic semialdehyde
dehydrogenase deficiency at just eight months old in 2011.
Catechol is oxidized to 2-hydroxymuconic semialdehyde
In the gastrodin-treated group, both succinic semialdehyde
dehydrogenase (SSADH) and succinic semialdehyde
reductase (SSAR) immunoreactivities in the hippocampus was also decreased significantly, which stood in contrast to the nontreated group, in which strong SSADH and SSAR immunoreactivities were detected.
GABA is converted to succinic semialdehyde
by GABA transaminase.
Jake, who attends Rowan Park special school in Litherland, has suffered with his condition, succinic semialdehyde
dehydrogenase deficiency, since he was a toddler.
In the metabolic process, glutamate generates GABA by the catalysis of GAD, and GABA generates succinic semialdehyde
by the catalysis of GABA-T (Zhu et al.
The GC-MS data also shows the presence of catechol and 2-hydroxymuconic semialdehyde
, which confirms the established pathway of benzene biodegradation.