Duchenne dystrophy: electron microscopic findings pointing to a basic or early abnormality in the plasma membrane of the muscle fiber

B. Mokri, Andrew G Engel

Research output: Contribution to journalArticle

395 Citations (Scopus)

Abstract

In seven patients with Duchenne dystrophy, high resolution phase microscopy demonstrated a population of non necrotic fibers with one or more focal lesions. The typical lesion was wedge shaped, with the base resting on the fiber surface. In the electron microscope, the plasma membrane overlying the lesion was either absent or disrupted, while the basement membrane was always preserved. Within the lesion, there were cytoplasmic abnormalities, and in the neighboring fiber region, the myofibrils were usually highly contracted. The structural defect in the plasma membrane suggested that this site was an ineffective cellular barrier. This was confirmed by the frequent ingress of peroxidase containing extracellular fluid into the lesions. In two control subjects, peroxidase penetration into fibers was seen only rarely and only with other evidence of mechanical injury to the specimen. The findings point to an early and possibly basic abnormality in the plasma membrane of the muscle fiber in Duchenne's dystrophy.

Original languageEnglish (US)
Pages (from-to)1111-1120
Number of pages10
JournalNeurology
Volume25
Issue number12
StatePublished - 1975

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Cell Membrane
Electrons
Muscles
Peroxidase
Myofibrils
Extracellular Fluid
Basement Membrane
Microscopy
Wounds and Injuries
Population

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Duchenne dystrophy : electron microscopic findings pointing to a basic or early abnormality in the plasma membrane of the muscle fiber. / Mokri, B.; Engel, Andrew G.

In: Neurology, Vol. 25, No. 12, 1975, p. 1111-1120.

Research output: Contribution to journalArticle

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