Apoptosis in metabolic myopathies

Maria C. Monici, Antonio Toscano, Paolo Girlanda, M'hammed Aguennouz, Olimpia Musumeci, Giuseppe Vita

Research output: Contribution to journalArticle

26 Citations (Scopus)

Abstract

DNA fragmentation, the hallmark of apoptosis, has been recently investigated with contradictory results in several skeletal muscle disorders. Using in situ labeling 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.

Original languageEnglish
Pages (from-to)2431-2435
Number of pages5
JournalNeuroReport
Volume9
Issue number10
Publication statusPublished - Jul 13 1998

Fingerprint

Muscular Diseases
Apoptosis
DNA Fragmentation
Multiple Acyl Coenzyme A Dehydrogenase Deficiency
Mitochondrial Myopathies
Deficiency Diseases
Mitochondrial Membrane Potential
Electron Transport
Permeability
Mitochondria
Skeletal Muscle
Muscles
Proteins

Keywords

  • Apoptosis
  • DNA fragmentation
  • Metabolic myopathies
  • Mitochondrial myopathies
  • TUNEL

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Monici, M. C., Toscano, A., Girlanda, P., Aguennouz, M., Musumeci, O., & Vita, G. (1998). Apoptosis in metabolic myopathies. NeuroReport, 9(10), 2431-2435.

Apoptosis in metabolic myopathies. / Monici, Maria C.; Toscano, Antonio; Girlanda, Paolo; Aguennouz, M'hammed; Musumeci, Olimpia; Vita, Giuseppe.

In: NeuroReport, Vol. 9, No. 10, 13.07.1998, p. 2431-2435.

Research output: Contribution to journalArticle

Monici, MC, Toscano, A, Girlanda, P, Aguennouz, M, Musumeci, O & Vita, G 1998, 'Apoptosis in metabolic myopathies', NeuroReport, vol. 9, no. 10, pp. 2431-2435.
Monici MC, Toscano A, Girlanda P, Aguennouz M, Musumeci O, Vita G. Apoptosis in metabolic myopathies. NeuroReport. 1998 Jul 13;9(10):2431-2435.
Monici, Maria C. ; Toscano, Antonio ; Girlanda, Paolo ; Aguennouz, M'hammed ; Musumeci, Olimpia ; Vita, Giuseppe. / Apoptosis in metabolic myopathies. In: NeuroReport. 1998 ; Vol. 9, No. 10. pp. 2431-2435.
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