Letellier 1992 Pediatr Res: Difference between revisions
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Latest revision as of 19:24, 17 April 2022
Letellier T, Malgat M, Coquet M,Moretto B, Parrot-Roulaud F, Mazat JP (1992) Mitochondrial myopathy studies on permeabilized muscle fibers. Pediatr Res 32: 17โ22. |
Letellier T, Malgat M, Coquet M, Moretto B, Parrot-Roulaud F, Mazat JP (1992) Pediatr Res
Abstract: Respiratory parameters of skeletal muscle were determined in permeabilized muscle fibers by adapting a technique described by Veksler et al. for cardiac fibers (Biochim Biophys Acta, 892:191-196, 1987). This method consists of the permeabilization of muscle fibers by saponin by allowing respiratory substrates and inhibitors to reach the mitochondria. In this way, the mitochondria may be studied inside the fibers as if they were isolated. We have verified, using various techniques, that the mitochondria remain intact during this procedure. This method has been applied to the study of six newborn infants for whom a diagnosis of a mitochondrial defect was suspected. In all cases, the defect was to be found on the permeabilized fibers, and this was confirmed by an enzymatic study. The advantage of this new method, associated with the measurement of the enzymatic activities on a crude homogenate, is to enable a simple and rapid diagnosis on a small amount of sample without damaging the mitochondria during the isolation procedure. โข Keywords: Mitochondrial myopathy
โข O2k-Network Lab: FR Bordeaux Letellier T
Cited by
- Komlodi et al (2022) Hydrogen peroxide production, mitochondrial membrane potential and the coenzyme Q redox state measured at tissue normoxia and experimental hyperoxia in heart mitochondria. MitoFit Preprints 2021 (in prep)
- Komlodi et al (2022) Hydrogen peroxide production, mitochondrial membrane potential and the coenzyme Q redox state measured at tissue normoxia and experimental hyperoxia in heart mitochondria. MitoFit Preprints 2021 (in prep)
Labels: MiParea: Respiration, mt-Medicine, Patients Pathology: Inherited
Organism: Human Tissue;cell: Skeletal muscle Preparation: Permeabilized tissue
Coupling state: OXPHOS
MitoFit 2021 Tissue normoxia