DNA topoisomerase II beta is shown to have an unsuspected and critical role
in neural development. Neurogenesis was normal in II beta mutant mice, but
motor axons failed to contact skeletal muscles, and sensory axons failed t
o enter the spinal cord. Despite an absence of innervation, clusters of ace
tylcholine receptors were concentrated in the central region of skeletal mu
scles, thereby revealing patterning mechanisms that are autonomous to skele
tal muscle. The defects in motor axon growth in II beta mutant mice resulte
d in a breathing impairment and death of the pups shortly after birth.