Antiquitin functions as a L-1-piperidine-6-carboxylate (P6C)/[alpha]-aminoadipic semialdehyde
The first step in the aerobic degradation pathway is oxidative deamination with the production of catechol, with further degradation to cis, cis-muconic acid by the catechol 1,2-dioxygenase (ortho-cleavage pathway) (Aoki et al., 1984; Loidl et al., 1990; Murakami et al., 2003) or to a yellow compound, 2-hydroxymuconic semialdehyde
by catechol 2,3-dioxygenase (the meta-cleavage pathway)(Fujii et al., 1997; Takeo et al., 1998a; Takeo et al., 1998b).
This disorder arises due to the mutation in the antiquitin gene which codes for the [alpha]-amino adipic semialdehyde
dehydrogenase enzyme that is responsible for the conversion of [alpha]-amino adipic semialdehyde
to [alpha]-amino adipic acid.
Among other features, the discovery of an RNA helicase and a glutamate semialdehyde
aminotransferase, which were still unknown in archaeal organisms, was reported.
(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.
During the reconstruction the authors also noted that shikimate production was incomplete and thus proposed that hexose and L-aspartate-4 semialdehyde
were used, consistent with [sup.13]C labeling data from tryptophan degradation.
Catechol can later be oxidized further to muconic acid or 2-hydroxymuconic semialdehyde
 which can finally be degraded to yield organic acids that are used by microorganisms for cell synthesis and energy production .
The loss of Thr and Lys could be due to the formation of intermediary adducts such as [alpha]-amino-3-keto butyric acid and [alpha]-amino adipic semialdehyde
, as a consequence of oxidation catalyzed by metals from the metalloproteins of the food matrix .
In the brain, tryptophan has two different metabolic fates: it can be metabolized into serotonin or it can enter the kynurenine pathway (KP) where it is degraded to [alpha]-amino-[beta]-carboxymuconate-[epsilon]- semialdehyde
(ACMS), which can be metabolized either into quinolinic acid for NAD synthesis or into 2-aminomuconate for entry into the TCA cycle.
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
. The Journal of Biological Chemistry.
A ketogenic diet rescues the murine succinic semialdehyde
dehydrogenase deficient phenotype.