Experimental Evidence that Methylmalonic Acid Provokes Oxidative Damage and Compromises Antioxidant Defenses in Nerve Terminal and Striatum of Young Rats

Glutaric aciduria type I and methylmalonic aciduria: Simulation of cerebral import and export of accumulating neurotoxic dicarboxylic acids in in vitro models of the blood–brain barrier and the choroid plexus

(2010) Sven W. Sauer et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Cysteine residues exposed on protein surfaces are the dominant intramitochondrial thiol and may protect against oxidative damage

(2010) Raquel Requejo et al.   FEBS Journal

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

Prevention by l-carnitine of DNA damage induced by propionic and l-methylmalonic acids in human peripheral leukocytes in vitro

(2010) Graziela S. Ribas et al.   MUTATION RESEARCH-GENETIC TOXICOLOGY AND ENVIRONMENTAL MUTAGENESIS

Prediction of outcome in isolated methylmalonic acidurias: combined use of clinical and biochemical parameters

(2009) F. Hörster et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Profiling of oxidative stress in patients with inborn errors of metabolism

(2009) Peter J. Mc Guire et al.   MOLECULAR GENETICS AND METABOLISM

Multiple OXPHOS Deficiency in the Liver, Kidney, Heart, and Skeletal Muscle of Patients With Methylmalonic Aciduria and Propionic Aciduria

(2009) Yves de Keyzer et al.   PEDIATRIC RESEARCH

Effect of selected antioxidants in β-cyfluthrin-induced oxidative stress in human erythrocytes in vitro

(2009) Izabela Sadowska-Woda et al.   TOXICOLOGY IN VITRO

Mitochondrial dysfunction in mut methylmalonic acidemia

(2008) Randy J. Chandler et al.   FASEB JOURNAL

Methylmalonate-induced seizures are attenuated in inducible nitric oxide synthase knockout mice

(2008) Leandro Rodrigo Ribeiro et al.   INTERNATIONAL JOURNAL OF DEVELOPMENTAL NEUROSCIENCE

Looking beyond the basal ganglia: The spectrum of MRI changes in methylmalonic acidaemia

(2008) I. Harting et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Relative efficacies of α-tocopherol, N-acetyl-serotonin, and melatonin in reducing non-enzymatic lipid peroxidation of rat testicular microsomes and mitochondria

(2008) Mariana Gavazza et al.   MOLECULAR AND CELLULAR BIOCHEMISTRY