ArticleJournal of inherited metabolic disease2007
Evidence for oxidative stress in tissues derived from succinate semialdehyde dehydrogenase-deficient mice.
Article in Journal of inherited metabolic disease, 2007. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers.
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Who cites it
20 citing papers in PubMed, 35 citations in OpenAlex.
- A Randomized Controlled Trial of SGS-742, a γ-aminobutyric acid B (GABA-B) Receptor Antagonist, for Succinic Semialdehyde Dehydrogenase Deficiency.Journal of child neurology · 2021Trial
- Transcriptomic profiling of long- and short-lived mutant mice implicates mitochondrial metabolism in ageing and shows signatures of normal ageing in progeroid mice.Mechanisms of ageing and development · 2021Article
- The effect of voluntary wheel running on the antioxidant status is dependent on sociability conditions.Pharmacology, biochemistry, and behavior · 2020Article
- SSADH Variants Increase Susceptibility of U87 Cells to Mitochondrial Pro-Oxidant Insult.International journal of molecular sciences · 2020Article
- Review
- Therapeutic relevance of mTOR inhibition in murine succinate semialdehyde dehydrogenase deficiency (SSADHD), a disorder of GABA metabolism.Biochimica et biophysica acta. Molecular basis of disease · 2017Article
- Succinic semialdehyde dehydrogenase deficiency (SSADHD): Pathophysiological complexity and multifactorial trait associations in a rare monogenic disorder of GABA metabolism.Neurochemistry international · 2016Review
- A case of acute onset succinic semialdehyde dehydrogenase deficiency: neuroimaging findings and literature review.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2016Review
- Natural history of succinic semialdehyde dehydrogenase deficiency through adulthood.Neurology · 2015Article
- Inherited disorders of gamma-aminobutyric acid metabolism and advances in ALDH5A1 mutation identification.Developmental medicine and child neurology · 2015Review
- Disorders of GABA metabolism: SSADH and GABA-transaminase deficiencies.Journal of pediatric epilepsy · 2014Article
- Defects in GABA metabolism affect selective autophagy pathways and are alleviated by mTOR inhibition.EMBO molecular medicine · 2014Article
- Taurine trial in succinic semialdehyde dehydrogenase deficiency and elevated CNS GABA.Neurology · 2014Article
- Evidence of redox imbalance in a patient with succinic semialdehyde dehydrogenase deficiency.Molecular genetics and metabolism reports · 2014Article
- Succinic semialdehyde dehydrogenase: biochemical-molecular-clinical disease mechanisms, redox regulation, and functional significance.Antioxidants & redox signaling · 2011Review
- Effects of 1,4-butanediol administration on oxidative stress in rat brain: study of the neurotoxicity of gamma-hydroxybutyric acid in vivo.Metabolic brain disease · 2009Article
- Neuroimaging findings in children with paediatric neurotransmitter diseases.Journal of inherited metabolic disease · 2009Review
- Redox-switch modulation of human SSADH by dynamic catalytic loop.The EMBO journal · 2009Article
- Comparative genomics of aldehyde dehydrogenase 5a1 (succinate semialdehyde dehydrogenase) and accumulation of gamma-hydroxybutyrate associated with its deficiency.Human genomics · 2009Review
- Neuropsychiatric morbidity in adolescent and adult succinic semialdehyde dehydrogenase deficiency patients.CNS spectrums · 2008Article
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Authors and funding
5 authors at 4 institutions in 2 countries.
Funding
Abstract
Animal models of inborn errors of metabolism are useful for investigating the pathogenesis associated with the corresponding human disease. Since the mechanisms involved in the pathophysiology of succinate semialdehyde dehydrogenase (SSADH) deficiency (Aldh5a1; OMIM 271980) are still not established, in the present study we evaluated the tissue antioxidant defences and lipid peroxidation in various cerebral structures (cortex, cerebellum, thalamus and hippocampus) and in the liver of SSADH-deficient mice. The parameters analysed were total radical-trapping antioxidant potential (TRAP) and glutathione (GSH) levels, the activities of the antioxidant enzymes superoxide dismutase (SOD), catalase (CAT) and glutathione peroxidase (GPx), as well as thiobarbituric acid-reactive substances (TBARS). We first observed that the tissue nonenzymatic antioxidant defences were significantly reduced in the SSADH-deficient animals, particularly in the liver (decreased TRAP and GSH) and in the cerebral cortex (decreased GSH), as compared to the wild-type mice. Furthermore, SOD activity was significantly increased in the liver and cerebellum, whereas the activity of CAT was significantly higher in the thalamus. In contrast, GPx activity was significantly diminished in the hippocampus. Finally, we observed that lipid peroxidation (TBARS levels) was markedly increased in the liver and cerebral cortex, reflecting a high lipid oxidative damage in these tissues. Our data showing an imbalance between tissue antioxidant defences and oxidative attack strongly indicate that oxidative stress is involved in the pathophysiology of SSADH deficiency in mice, and likely the corresponding human disorder.
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