Abstract
Focal lesions in the plasma membrane overlying wedge-shaped defects in muscle fibers ('delta lesions') are an early pathological change in Duchenne muscular dystrophy (DMD). Abnormalities in the plasma membrane have been suggested as a cause of these lesions and of the degeneration of muscle fibers in DMD. We investigated the role of plasma membrane defects in the production of delta lesions by examining the effects of a series of membrane-active agents - lysolecithin, deoxycholate, Triton X-100, and melittin - on the muscles of rats in vivo. Within minutes after treatment with these agents, the muscle fibers developed typical delta lesions. Identical morphological changes were produced by the calcium ionophore A23187, suggesting that calcium entry may play an important role in this process. We conclude that damage to the plasma membrane or calcium entry can reproduce characteristic features of the muscle pathology seen in DMD. This model should prove useful in elucidating the mechanisms of muscle fiber damage and degeneration in DMD.
| Original language | English (US) |
|---|---|
| Pages (from-to) | 209-214 |
| Number of pages | 6 |
| Journal | Muscle and Nerve |
| Volume | 5 |
| Issue number | 3 |
| State | Published - 1982 |
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ASJC Scopus subject areas
- Clinical Neurology
- Neuroscience(all)
Cite this
Membrane myopathy : Morphological similarities to Duchenne muscular dystrophy. / Pestronk, A.; Parhad, I. M.; Drachman, Daniel B; Price, D. L.
In: Muscle and Nerve, Vol. 5, No. 3, 1982, p. 209-214.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Membrane myopathy
T2 - Morphological similarities to Duchenne muscular dystrophy
AU - Pestronk, A.
AU - Parhad, I. M.
AU - Drachman, Daniel B
AU - Price, D. L.
PY - 1982
Y1 - 1982
N2 - Focal lesions in the plasma membrane overlying wedge-shaped defects in muscle fibers ('delta lesions') are an early pathological change in Duchenne muscular dystrophy (DMD). Abnormalities in the plasma membrane have been suggested as a cause of these lesions and of the degeneration of muscle fibers in DMD. We investigated the role of plasma membrane defects in the production of delta lesions by examining the effects of a series of membrane-active agents - lysolecithin, deoxycholate, Triton X-100, and melittin - on the muscles of rats in vivo. Within minutes after treatment with these agents, the muscle fibers developed typical delta lesions. Identical morphological changes were produced by the calcium ionophore A23187, suggesting that calcium entry may play an important role in this process. We conclude that damage to the plasma membrane or calcium entry can reproduce characteristic features of the muscle pathology seen in DMD. This model should prove useful in elucidating the mechanisms of muscle fiber damage and degeneration in DMD.
AB - Focal lesions in the plasma membrane overlying wedge-shaped defects in muscle fibers ('delta lesions') are an early pathological change in Duchenne muscular dystrophy (DMD). Abnormalities in the plasma membrane have been suggested as a cause of these lesions and of the degeneration of muscle fibers in DMD. We investigated the role of plasma membrane defects in the production of delta lesions by examining the effects of a series of membrane-active agents - lysolecithin, deoxycholate, Triton X-100, and melittin - on the muscles of rats in vivo. Within minutes after treatment with these agents, the muscle fibers developed typical delta lesions. Identical morphological changes were produced by the calcium ionophore A23187, suggesting that calcium entry may play an important role in this process. We conclude that damage to the plasma membrane or calcium entry can reproduce characteristic features of the muscle pathology seen in DMD. This model should prove useful in elucidating the mechanisms of muscle fiber damage and degeneration in DMD.
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M3 - Article
C2 - 7088018
AN - SCOPUS:0020050391
VL - 5
SP - 209
EP - 214
JO - Muscle and Nerve
JF - Muscle and Nerve
SN - 0148-639X
IS - 3
ER -