Succinic_semialdehyde

Succinic semialdehyde

Succinic semialdehyde

Chemical compound

Succinic semialdehyde (SSA) is a GABA metabolite. It is formed from GABA by the action of GABA transaminase (4-aminobutyrate aminotransferase) and further oxidised to become succinic acid, which enters TCA cycle. SSA is oxidized into succinic acid by the enzyme succinic semialdehyde dehydrogenase, which uses NAD+ as a cofactor.[2] [3]  When the oxidation of succinic semialdehyde to succinic acid is impaired, accumulation of succinic semialdehyde takes place which leads to succinic semialdehyde dehydrogenase deficiency.[3]

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References

  1. [1]
    Lide, David R. (1998), Handbook of Chemistry and Physics (87 ed.), Boca Raton, Florida: CRC Press, pp. 3–446, ISBN 0-8493-0594-2
  2. [2]
    Peng, Qi; Yang, Min; Wang, Wei; Han, Lili; Wang, Guannan; Wang, Pengyue; Zhang, Jie; Song, Fuping (2014-12-20). "Activation of gab cluster transcription in Bacillus thuringiensis by γ-aminobutyric acid or succinic semialdehyde is mediated by the Sigma 54-dependent transcriptional activator GabR". BMC Microbiology. 14 (1): 306. doi:10.1186/s12866-014-0306-3. ISSN 1471-2180. PMC 4279683. PMID 25527261.
  3. [3]
    Struys, E. A.; Jansen, E. E. W.; Gibson, K. M.; Jakobs, C. (December 2005). "Determination of the GABA analogue succinic semialdehyde in urine and cerebrospinal fluid by dinitrophenylhydrazine derivatization and liquid chromatography–tandem mass spectrometry: Application to SSADH deficiency". Journal of Inherited Metabolic Disease. 28 (6): 913–920. doi:10.1007/s10545-005-0111-0. ISSN 0141-8955. PMID 16435183. S2CID 9956364.


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