Apoptosis in metabolic myopathies
作者:
Maria Monici,
Antonio Toscano,
Paolo Girlanda,
M'hammed Aguennouz,
Olimpia Musumeci,
Giuseppe Vita,
期刊:
NeuroReport
(OVID Available online 1998)
卷期:
Volume 9,
issue 10
页码: 2431-2435
ISSN:0959-4965
年代: 1998
出版商: OVID
关键词: Apoptosis;DNA fragmentation;Metabolic myopathies;Mitochondrial myopathies;TUNEL
数据来源: OVID
摘要:
DNA fragmentation, the hallmark of apoptosis, has been recently investigated with contradictory results in several skeletal muscle disorders. Usingin situlabeling of nuclear DNA fragmentation, we have tested the possibility that apoptosis might occur in muscles from patients with mitochondrial respiratory chain defects and other types of metabolic myopathies. A high proportion of apoptotic myonuclei were found in all of 10 patients with mitochondrial myopathies and in one patient with multiple acyl-CoA dehydrogenase deficiency, a disease also affecting mitochondrial metabolism. These findings can be related to the intriguing link existing between apoptosis and mitochondria. It has been demonstrated that a fall of mitochondrial membrane potential constitutes a critical early event in the apoptotic process, and that mitochondrial bcl-2 protein, which protects from apoptosis, apparently functions as an endogenous permeability transition inhibitor.
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