Density, calibre and ramification of muscle capillaries are altered in a mouse model of severe spinal muscular atrophy

E Somers, Z Stencel, TM Wishart, TH Gillingwater… - Neuromuscular …, 2012 - Elsevier
Spinal muscular atrophy (SMA) is traditionally described and characterised as a disease of
the neuromuscular system. Recently, the vascular system has been implicated in SMA
pathogenesis, but there are no reports on whether this impacts on skeletal muscle
microvasculature. Using an established mouse model of severe SMA (Smn−/−; SMN2+/+),
we examined the capillary bed in three different skeletal muscles using quantitative imaging
and western blotting in late symptomatic mice (P5). We found a dramatic (45%) decrease in …