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. Author manuscript; available in PMC: 2017 Oct 1.
Published in final edited form as: Neurochem Int. 2016 Jun 14;99:72–84. doi: 10.1016/j.neuint.2016.06.009

Fig. 4. Pathophysiological alterations in heritable SSADH deficiency.

Fig. 4

Arrows indicate the direction and magnitude of metabolic disturbance. Abbreviations: 4-HNE, 4-hydroxyl-2-nonenal; GSH, glutathione; GluR, glutamate receptors; GABAAR/GABABR, GABAA and GABAB receptors; HC, homocarnosine (the dipeptide of GABA:L-histidine); GBA, guanidinobutyrate; Gln, glutamine. See Fig. 2 for other metabolic interconversions associated with GABA. 4-HNE is a major by-product of lipid peroxidation, and is metabolized by SSADH in brain. The dashed boxes indicate recent findings from the knockout mouse model which are not yet published, but are consistent with earlier reports in SSADHD (Pearl et al 2009; Reis et al 2012; Wu et al 2006; Buzzi et al 2006).