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Leptomeres in cultured human muscle
Acta Neuropathologica
|June 30, 1978
Summary
Leptomeres, abnormal laminated structures, were frequently found in muscle fibers of patients with certain myopathies. These structures can also be induced in normal muscle, suggesting a common cellular response to stress.
Area of Science:
- Cell Biology
- Neuromuscular Disorders
- Biochemistry
Background:
- Leptomeres are distinct laminated structures observed in muscle fibers.
- Their presence is often associated with specific myopathies, but their origin and induction are not fully understood.
Purpose of the Study:
- To investigate the occurrence of leptomeres in cultured muscle fibers from patients with various myopathies.
- To determine if leptomeres can be experimentally induced in normal muscle.
Main Methods:
- Culturing muscle fibers from patients with acid maltase deficiency, idiopathic "autophagic" vacuolar myopathy, and mitochondrial disorders.
- Examining cultured normal and denervated human muscle.
- Inducing leptomere formation in normal muscle cultures using 2,4-dinitrophenol (DNP).
Main Results:
- Leptomeres were frequently observed in muscle cultures from patients with acid maltase deficiency and idiopathic "autophagic" vacuolar myopathy.
- These structures were rare in normal and denervated muscle cultures.
- Exposure to 0.5 mM DNP abundantly induced leptomeres in normal human muscle cultures.
Conclusions:
- Leptomere formation is a characteristic feature in specific myopathies.
- The experimental induction of leptomeres in normal muscle suggests a common cellular mechanism or response pathway.
- Further research is needed to elucidate the precise role of leptomeres in muscle pathology.