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Maturational defect of regenerating muscle fibers in cases with duchenne and congenital muscular dystrophies
Author(s) -
Miike Teruhisa
Publication year - 1983
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.880060802
Subject(s) - duchenne muscular dystrophy , acridine orange , muscular dystrophy , anatomy , regeneration (biology) , fetus , mdx mouse , congenital muscular dystrophy , biology , pathology , medicine , skeletal muscle , dystrophin , microbiology and biotechnology , staining , pregnancy , genetics
Immature fibers in Duchenne muscular dystrophy (DMD) and congenital muscular dystrophies (CMD) were compared with human fetal muscles and experimentally‐induced regenerating muscle fibers using the acridine orange (AO) technique on fluorescent microscropy and histochemical methods. Strong AO activity was observed on immature fibers in dystrophies and on experimentally‐induced fibers in the early stages of regeneration. These young fibers showed type 2C histochemical reactions on ATPase stains. Human fetal muscles failed to show these fibers which were seen in the muscular dystrophies and the experimental regenerating muscle. From these results it is concluded that immature fibers in muscular dystrophies are early stages of regenerating fibers and not maturationally arrested fetal muscle fibers. In addition, these immature fibers formed small groups composed of 10 to 50 fibers in muscular dystrophies and sometimes large groups in CMD. It is suggested that the investigation of immature fibers and their grouping is quite important for revealing the pathogenesis of the muscular dystrophies.