Article
Decreased ATP synthesis is phenotypically expressed during increased energy demand in fibroblasts containing mitochondrial tRNA mutations.
European journal of biochemistry - 1 Jan 1999
James A M, Sheard P W, Wei Y H, Murphy M P
Abstract excerpt
Mutations in the tRNA genes of mitochondrial DNA (mtDNA) cause the debilitating MELAS (mitochondrial, myopathy, encephalopathy, lactic acidosis and stroke-like episodes) and MERRF (myoclonic epilepsy and ragged-red fibres) syndromes. These mtDNA mutations affect respiratory chain function, appare...
Topics
- Adenosine Diphosphate
- Adenosine Triphosphate
- Adolescent
- Adult
- Cell Death
- Cell Membrane
- Child, Preschool
- Fibroblasts
- Humans
- Intracellular Membranes
- MELAS Syndrome
- MERRF Syndrome
- Male
- Membrane Potentials
- Middle Aged
- Mitochondria
- Models, Biological
- Mutation