Bianca Seminotti

TL;DR: The present data indicate that KIC acts as an uncoupler of oxidative phosphorylation and as a metabolic inhibitor possibly through its inhibitory effect on alpha-ketoglutarate dehydrogenase activity, while Leu acts asA metabolic inhibitor.

TL;DR: The data indicate that Phe elicits oxidative stress in the hippocampus, a structure mainly involved with learning/memory, and also in the cerebral cortex, which is severely damaged in PKU patients, and it is presumed that this pathomechanism may be involved at least in part in the severe cognitive deficit and in the characteristic cortical atrophy observed in this disorder.

TL;DR: Investigation of in-vitro effects of MMA and PA on important parameters of lipid and protein oxidative damage and on the production of reactive species in synaptosomes from cerebrum of developing rats demonstrated the participation of neuronal cells in MMA-elicited oxidative damage.

TL;DR: The authors' data show a disruption of energy and redox homeostasis associated to inflammation induced by QUIN in the striatum of Gcdh−/− mice submitted to a high Lys diet, presumed that QUIN may possibly contribute to the pathophysiology of striatal degeneration in children with glutaric aciduria type I during inflammatory processes triggered by infections or vaccinations.

TL;DR: The data indicate that the metabolites accumulating in MGTA elicit oxidative stress in vitro in the cerebral cortex, and it is presumed that this pathomechanism may be involved in the brain damage observed in patients affected by MGTA.