Article
Axonal defects in mouse models of motoneuron disease.
Journal of neurobiology - 5 Feb 2004
Jablonka Sibylle, Wiese Stefan, Sendtner Michael
Abstract excerpt
Human motoneuron disease is characterized by loss of motor endplates, axonal degeneration, and cell death of motoneurons. The identification of the underlying gene defects for familial ALS, spinal muscular atrophy (SMA), and spinal muscular atrophy with respiratory distress (SMARD) has pointed to distinct pathophysiological mechanisms that are responsible for the various forms of the disease. Accumulating...
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