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Publication : The neuromuscular basis of hereditary kyphoscoliosis in the mouse.

First Author  Bridges LR Year  1992
Journal  Muscle Nerve Volume  15
Issue  2 Pages  172-9
PubMed ID  1372391 Mgi Jnum  J:116344
Mgi Id  MGI:3694050 Doi  10.1002/mus.880150208
Citation  Bridges LR, et al. (1992) The neuromuscular basis of hereditary kyphoscoliosis in the mouse. Muscle Nerve 15(2):172-9
abstractText  We describe a new neuromuscular disorder in the kyphoscoliotic mouse mutant (ky). Mice were killed at ages from birth to 210 days, and tissues were taken for standard light microscopy, histochemistry, nerve ending studies, and electron microscopy. At birth a few myofibers showed phagocytosis ultrastructurally. Between 6 and 25 days there was prominent necrosis and regeneration in soleus, gracilis, paraspinal, and back muscles. At 47 days, these muscles were atrophic and necrosis and regeneration were rare. At 136 days, all muscle groups, including head muscles, showed some degree of myofiber atrophy and gracilis was fibrotic. Prominent intramuscular axonal sprouting was present from 31 days. Peripheral nerves and anterior horn cells were normal. The findings indicate a neuromuscular basis of hereditary kyphoscoliosis in the mouse. The animal may be useful as a model of human muscle disease and scoliosis.
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