Article
Metabolic control analysis and mitochondrial pathologies.
Molecular and cellular biochemistry - 1 Jul 1998
Letellier T, Malgat M, Rossignol R, Mazat J P
Abstract excerpt
One of the main salient features recognized in mitochondrial diseases is the existence of a threshold in the degree of a mitochondrial deficit for the expression of the disease. When expressed as a function of the degree of heteroplasmy, the value of the threshold can be very high, around 90% (mu...
Topics
- Cell Respiration
- DNA, Mitochondrial
- Electron Transport Complex IV
- Energy Metabolism
- Humans
- Hybrid Cells
- Lymphocytes
- Mitochondria
- Mitochondrial ADP, ATP Translocases
- Mutation
- Oxygen Consumption
- Potassium Cyanide
- Protein Biosynthesis
- Sequence Deletion
- Transcription, Genetic
