Duchenne dystrophy. II. Morphometric study of motor end plate fine structure

F. Jerusalem, Andrew G Engel, M. R. Gomez

Research output: Contribution to journalArticle

36 Citations (Scopus)

Abstract

The fine structure of 36 peroneus brevis motor end plates from 3 cases of Duchenne dystrophy was quantitatively analyzed. The findings were compared with those in 32 external intercostal end plates from 13 control adults and 13 peroneus brevis end plates of a control boy. The mean nerve terminal area, the mitochondrial area per nerve terminal, the synaptic vesicle count per unit nerve terminal area and the synaptic vesicle diameter are normal in Duchenne dystrophy. Degenerating nerve terminals or intramuscular nerve fibers were not observed in dystrophic muscle. The consistent abnormality in the disease is focal atrophy of the postsynaptic folds. This is associated with a significant decrease in the postsynaptic membrane profile concentration and in the ratio of the postsynaptic to the presynaptic membrane length. Six of 75 postsynaptic regions appeared denuded of their nerve terminal in the plane of the section but adjacent end plate regions on the same fibers where this was observed remained innervated. A normal nerve terminal was associated with a neuromuscular junction situated on a necrotic fiber. In 24 end plate bearing muscle fibers of the dystrophic patients the mean mitochondrial surface density was significantly greater than in 33 muscle fibers of non weak children.

Original languageEnglish (US)
Pages (from-to)123-130
Number of pages8
JournalBrain
Volume97
Issue number1
StatePublished - 1974

Fingerprint

Motor Endplate
Plate Structures
Fine Structure
Nerve
nerve tissue
Synaptic Vesicles
Muscles
Fibers
Muscle
Fiber
Membranes
Neuromuscular Junction
Nerve Fibers
muscle fibers
Bearings (structural)
Vesicles
Atrophy
Membrane
nerve fibers
atrophy

ASJC Scopus subject areas

  • Neuroscience(all)
  • Statistics, Probability and Uncertainty
  • Applied Mathematics
  • Mathematics(all)
  • Statistics and Probability
  • Agricultural and Biological Sciences (miscellaneous)
  • Clinical Neurology

Cite this

Duchenne dystrophy. II. Morphometric study of motor end plate fine structure. / Jerusalem, F.; Engel, Andrew G; Gomez, M. R.

In: Brain, Vol. 97, No. 1, 1974, p. 123-130.

Research output: Contribution to journalArticle

Jerusalem, F, Engel, AG & Gomez, MR 1974, 'Duchenne dystrophy. II. Morphometric study of motor end plate fine structure', Brain, vol. 97, no. 1, pp. 123-130.
Jerusalem, F. ; Engel, Andrew G ; Gomez, M. R. / Duchenne dystrophy. II. Morphometric study of motor end plate fine structure. In: Brain. 1974 ; Vol. 97, No. 1. pp. 123-130.
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