P. Heimann et al., OVERSHOOTING PRODUCTION OF SATELLITE CELLS IN MURINE SKELETAL-MUSCLE AFFECTED BY THE MUTATION MUSCULAR-DYSTROPHY WITH MYOSITIS (MDM, CHR-2), Cell and tissue research, 283(3), 1996, pp. 435-441
The hereditary degenerative muscle disease ''muscular dystrophy with m
yositis'' of the mouse (MDM, genotype mdm/mdm) is caused by a spontane
ous recessive mutation on chromosome 2. Skeletal muscles of MDM mice d
egenerate during the first few postnatal weeks and subsequently regene
rate. 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 demon
strate a transient increase of up to 4-fold in the satellite cell freq
uency in both fast and slow muscles of affected animals. This differen
ce from wildtype controls (+/+ or +/mdm) is also reflected in the yiel
d of mononucleate myogenic cells upon dissociation of skeletal muscle
and subsequent cell culture. Unlike the increase in satellite cell den
sity in the myotonic ADR mouse, this phenomenon is not accompanied wit
h a shift to more oxidative fibre types. It may however be related to
the more profuse micro-vascularization of MDM muscle.