Article
Myoclonic epilepsy and ragged red fibers (MERRF) syndrome: selective vulnerability of CNS neurons does not correlate with the level of mitochondrial tRNAlys mutation in individual neuronal isolates.
The Journal of neuroscience : the official journal of the Society for Neuroscience - 15 Oct 1997
Zhou L, Chomyn A, Attardi G, Miller C A
Abstract excerpt
Selective vulnerability of subpopulations of neurons is a striking feature of neurodegeneration. Mitochondrially transmitted diseases are no exception. In this study CNS tissues from a patient with myoclonus epilepsy and ragged red fibers (MERRF) syndrome, which results from an A to G transition...
Topics
- Adolescent
- Cell Count
- Cell Death
- Cell Separation
- Central Nervous System
- Female
- Humans
- MERRF Syndrome
- Mutation
- Neurons
- Purkinje Cells
- RNA
- RNA, Mitochondrial
- RNA, Transfer