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Myotonia congenita. A histochemical and ultrastructural study in the goat: comparison with abnormalities found in human myotonia dystrophica.
- Source :
-
The American journal of pathology [Am J Pathol] 1981 Mar; Vol. 102 (3), pp. 324-35. - Publication Year :
- 1981
-
Abstract
- Muscle biopsy specimens from the myotonic goat, an animal model of heritable myotonia, were examined histochemically and by electron microscopy. After Periodic acid-Schiff (PAS) staining with diastase digestion, there was increased PAS-positive material within myotonic goat fibers, as compared with those of normal goats. Myotonic muscle stained with alizarin red S, a histochemical stain for calcium, also had an increased staining reaction when compared with muscle from normal goats. Several ultrastructural abnormalities were found in myotonic goat muscle using routine osmium and uranyl acetate staining. These included increased density of the t-tubules, electron-dense material within t-tubules, proliferation and dilatation of sarcotubular elements, and abnormal mitochondria in the myotonic biopsy specimens. To further study muscle ultrastructure, ruthenium red and lanthanum were used as electron microscopic stains with specificity for membranes. There was increased density of the sarcolemma and t-tubules in myotonic muscle stained with ruthenium red as compared to normal, and lanthanum produced a darker staining reaction of the myotonic goat sarcolemma. The histochemical and ultrastructural differences between normal and myotonic goat muscle were interpreted to be consistent with a morphologic basis for the abnormal contraction-relaxation properties characteristic of myotonia.
- Subjects :
- Adolescent
Adult
Animals
Calcium analysis
Cell Fractionation
Disease Models, Animal
Female
Goats
Histocytochemistry
Humans
Male
Mitochondria analysis
Mitochondria ultrastructure
Muscles analysis
Muscles ultrastructure
Myotonia Congenita pathology
Myotonia Congenita veterinary
Myotonic Dystrophy veterinary
Sarcolemma ultrastructure
Muscles pathology
Myotonic Dystrophy pathology
Subjects
Details
- Language :
- English
- ISSN :
- 0002-9440
- Volume :
- 102
- Issue :
- 3
- Database :
- MEDLINE
- Journal :
- The American journal of pathology
- Publication Type :
- Academic Journal
- Accession number :
- 7212017