Neurochemical Evidence that the Metabolites Accumulating in 3-Methylcrotonyl-CoA Carboxylase Deficiency Induce Oxidative Damage in Cerebral Cortex of Young Rats

Disruption of redox homeostasis in cerebral cortex of developing rats by acylcarnitines accumulating in medium-chain acyl-CoA dehydrogenase deficiency

(2012) Anelise M. Tonin et al.   INTERNATIONAL JOURNAL OF DEVELOPMENTAL NEUROSCIENCE

A single mutation in MCCC1 or MCCC2 as a potential cause of positive screening for 3-methylcrotonyl-CoA carboxylase deficiency

(2012) Raphael J. Morscher et al.   MOLECULAR GENETICS AND METABOLISM

3-methylcrotonyl-CoA carboxylase deficiency: Clinical, biochemical, enzymatic and molecular studies in 88 individuals

(2012) Sarah C Grünert et al.   Orphanet Journal of Rare Diseases

3-Methylcrotonylglycine Disrupts Mitochondrial Energy Homeostasis and Inhibits Synaptic Na+,K+-ATPase Activity in Brain of Young Rats

(2011) Alana Pimentel Moura et al.   CELLULAR AND MOLECULAR NEUROBIOLOGY

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

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

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

Novel mutations in the human MCCA and MCCB gene causing methylcrotonylglycinuria

(2010) Khue Vu Nguyen et al.   MOLECULAR GENETICS AND METABOLISM

Neurochemical Evidence that Lysine Inhibits Synaptic Na+,K+-ATPase Activity and Provokes Oxidative Damage in Striatum of Young Rats In vivo

(2010) Bianca Seminotti et al.   NEUROCHEMICAL RESEARCH

Mitochondria and reactive oxygen species

(2009) Alicia J. Kowaltowski et al.   FREE RADICAL BIOLOGY AND MEDICINE

Protein carbonylation: 2,4-dinitrophenylhydrazine reacts with both aldehydes/ketones and sulfenic acids

(2009) Isabella Dalle-Donne et al.   FREE RADICAL BIOLOGY AND MEDICINE

Hyperglycemia-induced alterations in synaptosomal membrane fluidity and activity of membrane bound enzymes: beneficial effect of N-acetylcysteine supplementation

(2009) S. Singh Kamboj et al.   NEUROSCIENCE

Optimization and validation of an alternative method to evaluate total reactive antioxidant potential

(2008) Maria T.K. Dresch et al.   ANALYTICAL BIOCHEMISTRY

Induction of oxidative stress by the metabolites accumulating in 3-methylglutaconic aciduria in cerebral cortex of young rats

(2008) Guilhian Leipnitz et al.   LIFE SCIENCES

Recurrent attacks of status epilepticus as predominant symptom in 3-methylcrotonyl-CoA carboxylase deficiency

(2007) Eray Dirik et al.   BRAIN & DEVELOPMENT

A Delphi-based consensus clinical practice protocol for the diagnosis and management of 3-methylcrotonyl CoA carboxylase deficiency

(2007) Georgianne L. Arnold et al.   MOLECULAR GENETICS AND METABOLISM