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Histopathological characterization of the skeletal myopathy in rasH2 mice carrying human prototype c-Ha-ras gene
Takayuki Tsuchiya1, Miyoko Okada, Tetsuya Sakairi
1Toxicology Laboratory, Pharmaceuticals Research Center, Mitsubishi Pharma Corporation, 1-1-1 Kazusakamatari, Kisarazu, Chiba 292-0818, Japan.
Abstract:
A skeletal myopathy is found in approximately 100% of rasH2 mice. To confirm detailed features of the rasH2 skeletal myopathy, the biceps femoris, diaphragm, triceps brachii, gastrocnemial (types I and II fiber-mixed muscles) and soleus muscle (type I fiber-dominant muscle) obtained from male rasH2 and non-transgenic littermates aged 10-13 and 34 weeks were examined. Variations in the muscle fiber size, early-scattered degeneration/necrosis and regeneration of muscle fibers were detected in 10-13-week-old rasH2 mice. The severity of the above muscular lesions was more prominent in older rasH2 mice. These lesions were noted in the type II myofiber dominant muscles (biceps femoris, triceps brachii and gastrocnemial). NADH-TR stain clearly demonstrated a disorganized intermyofibrillar network and necrotic change in muscle fibers. No specific morphological changes, like rod structure or tubular aggregation seen in some types of myopathy, were noted in Gomori trichrome and NADH-TR stains in the rasH2 mouse like in many types of muscular dystrophy. Electronmicroscopically, occasional muscle fiber degeneration/regeneration, invaded phagocytic cells, indistinct Z-band suggesting excessive contraction and dilatation of the sarcoplasmic reticulum were observed. In summary, the skeletal myopathy occurring in rasH2 mice is consistent with muscular dystrophy characterized morphologically by progressive degeneration and regeneration of myofibers. The myopathy is confined to the type II myofiber predominant muscles and is not associated with any pathognomonic lesions. These characteristics will provide us with a useful model for research in muscular dystrophy of diverse myofibers.
Insights
RasH2 mice exhibit skeletal myopathy resembling muscular dystrophy, primarily affecting type II muscle fibers. This provides a valuable model for studying progressive muscle degeneration and regeneration in diverse myofiber types.
Area of Science:
- Genetics and Molecular Biology
- Pathology
- Animal Models
Background:
- RasH2 mice spontaneously develop skeletal myopathy.
- Understanding the specific characteristics of this myopathy is crucial for its use as a research model.
Purpose of the Study:
- To detail the morphological features of skeletal myopathy in rasH2 mice.
- To evaluate the suitability of rasH2 mice as a model for muscular dystrophy research.
Main Methods:
- Histopathological examination of various muscles (biceps femoris, diaphragm, triceps brachii, gastrocnemial, soleus) from rasH2 and control mice at different ages.
- Histochemical staining (NADH-TR, Gomori trichrome).
- Electron microscopy.
Main Results:
- RasH2 mice showed progressive skeletal myopathy with variations in muscle fiber size, degeneration, necrosis, and regeneration, predominantly in type II myofiber muscles.
- Histochemical and electron microscopic analyses revealed disorganized muscle structure and degeneration/regeneration, consistent with muscular dystrophy.
- No specific pathognomonic lesions like rod structures were observed.
Conclusions:
- The skeletal myopathy in rasH2 mice is morphologically consistent with muscular dystrophy, characterized by progressive myofiber degeneration and regeneration.
- The myopathy is confined to type II myofiber predominant muscles and lacks unique pathological markers.
- RasH2 mice represent a useful model for investigating muscular dystrophies affecting diverse myofiber types.

