Glutaric acidemia type 1 (GA I) is caused by deficient activity of glutaryl-CoA dehydrogenase, leading to predominant accumulation of glutaric acid (GA) in the brain. GA I patients present progressive neurological deterioration whose pathophysiology is only partially elucidated. We investigated whether intracerebral GA...
E. T. Castro, Â. Zanatta, A. Carvalho et al.· Metabolic brain disease· 0 citations
X-linked adrenoleukodystrophy (X-ALD) is the most common peroxisomal disorder. Although the quantification of very long-chain fatty acids (VLCFA) is an old technique implemented to diagnose and monitor, the limited number of experienced clinical biochemists in this field and also the limited availability of technical r...
Ana Kalise Böttcher, Ana Luiza da Silveira Pereira, Débora Tonelotto et al.· Clinica chimica acta; intern...· 0 citations
Background/Objectives: High-protein (HP) diets may influence body composition, lipid metabolism, oxidative stress, and mitochondrial function, but their interaction with probiotics remains unclear. This study evaluated the effects of HP feeding, alone or combined with Lactobacillus acidophilus and Bifidobacterium lacti...
Luiza Ferracini Cunha, Isabella Pompeu Mattos Almeida, Andrew Oliveira Silva et al.· Nutrients· 0 citations
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