Heimann P, Menke A, Rothkegel B, Jockusch H
Developmental Biology Unit, University of Bielefeld, Germany.
Cell Tissue Res. 1996 Mar;283(3):435-41. doi: 10.1007/s004410050554.
The hereditary degenerative muscle disease "muscular dystrophy with myositis" of the mouse (MDM, genotype mdm/mdm) is caused by a spontaneous recessive mutation on chromosome 2. Skeletal muscles of MDM mice degenerate during the first few postnatal weeks and subsequently regenerate. The cellular and molecular mechanisms of this muscle disease are not yet understood. An inflammatory component as implied by its name seems unlikely. By applying quantitative electron microscopy, we demonstrate a transient increase of up to 4-fold in the satellite cell frequency in both fast and slow muscles of affected animals. This difference from wildtype controls (+/+ or +/mdm) is also reflected in the yield of mononucleate myogenic cells upon dissociation of skeletal muscle and subsequent cell culture. Unlike the increase in satellite cell density in the myotonic ADR mouse, this phenomenon is not accompanied with a shift to more oxidative fibre types. It may however be related to the more profuse micro-vascularization of MDM muscle.
小鼠的遗传性退行性肌肉疾病“伴肌炎的肌营养不良”（MDM，基因型mdm/mdm）由2号染色体上的自发隐性突变引起。MDM小鼠的骨骼肌在出生后的头几周内退化，随后再生。这种肌肉疾病的细胞和分子机制尚不清楚。从其名称来看，炎症成分似乎不太可能。通过应用定量电子显微镜，我们证明了受影响动物的快肌和慢肌中卫星细胞频率短暂增加高达4倍。与野生型对照（+/+或+/mdm）的这种差异也反映在骨骼肌解离并随后进行细胞培养后单核生肌细胞的产量上。与强直性ADR小鼠中卫星细胞密度的增加不同，这种现象并不伴随着向更多氧化型纤维类型的转变。然而，它可能与MDM肌肉中更丰富的微血管化有关。
