Mitochondrial energy metabolism in neurodegeneration associated with methylmalonic acidemia

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

Ion-abrasion scanning electron microscopy reveals distorted liver mitochondrial morphology in murine methylmalonic acidemia

(2010) Gavin E. Murphy et al.   JOURNAL OF STRUCTURAL BIOLOGY

Mitochondria and reactive oxygen species

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

Multiple OXPHOS deficiency in the liver of a patient with CblA methylmalonic aciduria sensitive to vitamin B12

(2009) V. Valayannopoulos et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Long-term exposure of human proximal tubule cells to hydroxycobalamin[c-lactam] as a possible model to study renal disease in methylmalonic acidurias

(2009) S. W. Sauer et al.   JOURNAL OF INHERITED METABOLIC DISEASE

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

Impact of short- and medium-chain organic acids, acylcarnitines, and acyl-CoAs onmitochondrial energy metabolism

(2008) Sven Wolfgang Sauer et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Mitochondrial dysfunction in mut methylmalonic acidemia

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

Methylmalonate inhibits succinate-supported oxygen consumption by interfering with mitochondrial succinate uptake

(2008) S. R. Mirandola et al.   JOURNAL OF INHERITED METABOLIC DISEASE

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

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