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Ultrastructural and histochemical features of skeletal muscle in the Wohlfart-Kugelberg-Welander syndrome

Authors
Journal
Journal of the Neurological Sciences
0022-510X
Publisher
Elsevier
Publication Date
Volume
13
Issue
1
Identifiers
DOI: 10.1016/0022-510x(71)90204-8
Disciplines
  • Chemistry

Abstract

Abstract Correlated electron-microscopic and histochemical studies of skeletal muscle biopsies from 2 patients with the Wohlfart-Kugelberg-Welander syndrome revealed the following morphological changes during the course of atrophy: (1) focal degeneration of I bands; (2) dissolution of Z lines, accompanied by an increase in number and size of mitochondria; (3) loss of entire sarcomeres, and narrowing of myofibrils. These observations have been compared with previous reports of the fine structure of skeletal muscle in amyotrophic lateral sclerosis and progressive spinal muscular atrophy, as well as in experimental denervation atrophy in various species.

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