Increased oxidative stress in patients with 3-hydroxy-3-methylglutaric aciduria

Redox homeostasis is compromised in vivo by the metabolites accumulating in 3-hydroxy-3-methylglutaryl-CoA lyase deficiency in rat cerebral cortex and liver

(2013) M. S. da Rosa et al.   FREE RADICAL RESEARCH

l-carnitine supplementation as a potential antioxidant therapy for inherited neurometabolic disorders

(2013) Graziela S. Ribas et al.   GENE

In vivo experimental evidence that the major metabolites accumulating in 3-hydroxy-3-methylglutaryl-CoA lyase deficiency induce oxidative stress in striatum of developing rats: A potential pathophysiological mechanism of striatal damage in this disorder

(2013) Carolina Gonçalves Fernandes et al.   MOLECULAR GENETICS AND METABOLISM

Urinary biomarkers of oxidative status

(2012) Dora Il'yasova et al.   CLINICA CHIMICA ACTA

Protein and lipid damage in maple syrup urine disease patients: l-carnitine effect

(2012) Caroline Paula Mescka et al.   INTERNATIONAL JOURNAL OF DEVELOPMENTAL NEUROSCIENCE

Oxidative Stress Parameters in Urine from Patients with Disorders of Propionate Metabolism: a Beneficial Effect of l-Carnitine Supplementation

(2011) Graziela S. Ribas et al.   CELLULAR AND MOLECULAR NEUROBIOLOGY

In vivo neuroprotective effect of L-carnitine against oxidative stress in maple syrup urine disease

(2011) Caroline Mescka et al.   METABOLIC BRAIN DISEASE

Evidence that l-Carnitine and Selenium Supplementation Reduces Oxidative Stress in Phenylketonuric Patients

(2010) A. Sitta et al.   CELLULAR AND MOLECULAR NEUROBIOLOGY

Reduction of lipid and protein damage in patients with disorders of propionate metabolism under treatment: a possible protective role of l-carnitine supplementation

(2010) Graziela S. Ribas et al.   INTERNATIONAL JOURNAL OF DEVELOPMENTAL NEUROSCIENCE

Neurochemical evidence that 3-methylglutaric acid inhibits synaptic Na+,K+-ATPase activity probably through oxidative damage in brain cortex of young rats

(2010) César Augusto João Ribeiro et al.   INTERNATIONAL JOURNAL OF DEVELOPMENTAL NEUROSCIENCE

Déficit en 3-HMG-CoA lyase à révélation tardive : savoir reconnaître une maladie rare mais traitable

(2009) S. Pierron et al.   ARCHIVES DE PEDIATRIE

Striatum is more vulnerable to oxidative damage induced by the metabolites accumulating in 3-hydroxy-3-methylglutaryl-CoA lyase deficiency as compared to liver

(2009) Guilhian Leipnitz et al.   INTERNATIONAL JOURNAL OF DEVELOPMENTAL NEUROSCIENCE

l-Carnitine Blood Levels and Oxidative Stress in Treated Phenylketonuric Patients

(2008) Angela Sitta et al.   CELLULAR AND MOLECULAR NEUROBIOLOGY

Selective screening for organic acidemias by urine organic acid GC–MS analysis in Brazil: Fifteen-year experience

(2008) Moacir Wajner et al.   CLINICA CHIMICA ACTA

Evidence that 3-hydroxy-3-methylglutaric acid promotes lipid and protein oxidative damage and reduces the nonenzymatic antioxidant defenses in rat cerebral cortex

(2007) Guilhian Leipnitz et al.   JOURNAL OF NEUROSCIENCE RESEARCH