engleski

The role of programmed cell death in Werdnig-Hoffmann disease

The development of the vertebrate nervous system is characterized by an initial overproduction of neurons in many regions followed by large-scale elimination. It is esimated that, immediately following the arrival of their axons in muscle cells, about 40-70% of the embryonic motor neurons in the spinal cord undergo naturally occurring programmed cell death during the midgestational period (15-25 weeks of gestation in the human fetus). The hypothesis that spinal muscular atrophies, particularly Werdnig-Hoffmann disease or SMA type-1, is based on an inappropriate persistence of normally occurring apoptosis is presented and discussed with respect to recent genetic and neuropathological results from our and other laboratories.

spinal muscular atrophy; pathogenesis; apoptosis

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