Mitochondrial Myopathy Studies on Permeabilized Muscle Fibers1
作者:
THIERRY,
LETELLIER MONIQUE,
MALGAT MICHELLE,
COQUET BRIGITTE,
MORETTO FRANÇOISE,
PARROT-ROULAUD JEAN-PIERRE,
期刊:
Pediatric Research
(OVID Available online 1992)
卷期:
Volume 32,
issue 1
页码: 17-22
ISSN:0031-3998
年代: 1992
出版商: OVID
数据来源: OVID
摘要:
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.
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