glutarylcarnitine has been researched along with Disease-Models--Animal* in 1 studies
1 other study(ies) available for glutarylcarnitine and Disease-Models--Animal
| Article | Year |
|---|---|
l-Carnitine prevents oxidative stress in striatum of glutaryl-CoA dehydrogenase deficient mice submitted to lysine overload.
The deficiency of the enzyme glutaryl-CoA dehydrogenase leads to predominant accumulation of glutaric acid (GA) in the organism and is known as glutaric acidemia type I (GA1). Despite the mechanisms of brain damage involved in GA1 are not fully understood, oxidative stress may be involved in this process. Treatment is based on protein/lysine (Lys) restriction and l-carnitine (L-car) supplementation. L-car was recently shown to have an important antioxidant role. A knockout mice model (Gcdh Topics: Amino Acid Metabolism, Inborn Errors; Animals; Brain Diseases, Metabolic; Carnitine; Corpus Striatum; Diet; Disease Models, Animal; Glutaryl-CoA Dehydrogenase; Glutathione Peroxidase; Lysine; Mice; Mice, Knockout; Oxidative Stress; Reactive Oxygen Species; Superoxide Dismutase | 2019 |