Abstract
In this article I illustrated the use of regenerating human muscle cultures for studying the hereditary human myopathies. Although some of the data are still controversial, they do point up the great potential of this "in vitro system". For hereditary myopathies due to developmentally regulated proteins that are expressed only at a more advanced stage of muscle differentiation, the use of highly differentiated nerve-muscle cocultures might contribute significantly to a better understanding of their developmental pathogenesis. More advanced techniques (permanent human muscle cell lines, heterokaryons, myoblast transfer, gene transfer, myogenic conversion of human non-muscle cells, cybrid clones) may provide a great deal of information at molecular level and may also have practical applications in the diagnosis or even in the treatment of hereditary human myopathies.
Original language | English |
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Pages (from-to) | 257-268 |
Number of pages | 12 |
Journal | Italian Journal of Neurological Sciences |
Volume | 12 |
Issue number | 3 |
DOIs | |
Publication status | Published - Jun 1991 |
Keywords
- cell lines
- clones
- cybrid clones
- gene transfer
- hereditary human myopathies
- heterokaryons
- Human muscle cultures
- Myo D
- myoblast transfer
- nerve-muscle cocultures
ASJC Scopus subject areas
- Neuroscience(all)
- Clinical Neurology