ALDH5A1

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ALDH5A1

A gene on chromosome 6p22 that encodes mitochondrial matrix NAD-dependent succinic semialdehyde dehydrogenase, of the aldehyde dehydrogenase family of proteins.

Molecular pathology
ALDH5A1 mutation causes 4-hydroxybutyricaciduria, which, due to an inability to metabolise GABA, results in accumulation of GHB, a neuromodulator.
References in periodicals archive ?
M2 PHARMA-October 25, 2017-Aldeyra Therapeutics Data Show ADX-102 Reduces GHB and GABA in Mouse Model of SSADH Deficiency
A small number of patients with a diagnosis of autism have been found to have SSADH deficiency.
The data presented showed analyses of GHB and GABA levels in the brains of SSADH knockout mice.
Later, a more complete in vivo study evidenced that gastrodin isolated from the same extract was able to decrease the immunoreactivities of the three enzymes involved in GABA degradation (GABA-T, SSADH and succinic semialdehyde reductase (SSAR)] (Table 2, entry 8; Fig.
Furthermore, structure-activity relationship analysis on 4-hydroxybenzaldehyde derivatives as GABA-T and SSADH inhibitors indicated that a carbonyl or an amino group as well as a hydroxyl group at the para-position of the benzene rings are important for the inhibition of both enzymes.
Therefore, several antiseizure mechanisms of action studied are oriented towards the involvement of these neurotransmitters, particularly the inhibition of enzymes involved in the degradation of GABA: GABA-T, SSADH and SSAR.
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.
2 [micro]M, and scutellarein exhibited the best inhibitory effect on SSADH with an [IC.
In fact, the administration of GABA-T and SSADH inhibitors such as vigabatrin (Valdizan et al.
breviscapus extract and its flavonoid components including scutellarin on GABA and SSADH, and have described the structure-activity relationships of these compounds, in order to develop new therapeutic applications of E.
Mike Gibson, PhD, is a world-renowned expert and researcher on SSADH.
Sam did not respond to medication commonly used in SSADH but did respond to physical, occupational, and speech therapies.