Article
Activation of the mitochondrial protein quality control system and actin cytoskeletal alterations in cells harbouring the MELAS mitochondrial DNA mutation.
Journal of the neurological sciences - 15 Aug 2010
Felk Sandra, Ohrt Susanne, Kussmaul Lothar, Storch Alexander, Gillardon Frank
Abstract excerpt
Point mutations in the mitochondrial genome are associated with a variety of metabolic disorders. The myopathy, encephalopathy, lactic acidosis, stroke-like episodes syndrome (MELAS), is most frequently associated with an A to G transition at position 3243 of the mitochondrial tRNA(Leu(UUR)) gene, and is characterized by biochemical and structural alterations of mitochondria. In the present study, we analyzed...
Topics
- Actins
- Cytoskeleton
- DNA, Mitochondrial
- Electrophoresis, Gel, Two-Dimensional
- Humans
- Lymphocytes
- MELAS Syndrome
- Mass Spectrometry
- Membrane Potential, Mitochondrial
- Mitochondrial Proteins
- Mutation
- Proteome