DYSTROPHIA MUSCULARIS - A HEREDITARY PRIMARY MYOPATHY IN THE HOUSE MOUSE
DYSTROPHIA MUSCULARIS - A HEREDITARY PRIMARY MYOPATHY IN THE HOUSE MOUSE
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DOI：
10.1073/pnas.41.12.1079
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发表时间：
1955-01-01
影响因子：
11.1
通讯作者：
HARMAN, PJ
中科院分区：
文献类型：
--
作者：
MICHELSON, AM;RUSSELL, ES;HARMAN, PJ
A primary myopathic mutation, characterized by progressive ataxia, atrophy, and paralysis, occurred in a colony of inbred strain 129 mice in 1951. It has been suggested that the hereditary anomaly be referred to as dystrophia muscularis, designated by the symbol dy. Clinical manifestations include consistent lightness of body weight, general atrophy of the axial and limb muscles, convulsive nodding of the head, kyphosis, paresis accompanied by spasmodic flexion and flaccid extension of the hind limbs, eventual complete loss of locomotor function in that region, and premature death. Breeding data from the pedigree of the strain 129 colony and from ovarian transplantation experiments are interpreted as indicating the presence of a single autosomal gene, whose full recessive expression of 25% is obscured to a maximal average of 21%. Microscopic study revealed the pathological involvement of muscular tissue and the absence of ostensible lesions of central as well as of peripheral nervous tissue which are characteristic of the recognized histopathological syndrome of muscular dystrophy. The mutant should provide a useful tool for research, since it is apparently the 1st experimental animal of known genetic background to present a myopathy similar to that found in the human muscular dystrophies.
