Neurogenic and Myogenic Contributions to Hereditary Motor Neuron Disease

作者：Bricceno Katherine V; Fischbeck Kenneth H; Burnett Barrington G^*

来源：Neurodegenerative Diseases, 2012, 9(4): 199-209.

DOI：10.1159/000335311

• Hereditary motor neuron disease
• Spinal muscular atrophy
• Spinal and bulbar muscular atrophy

Spinal muscular atrophy and spinal and bulbar muscular atrophy are characterized by lower motor neuron loss and muscle atrophy. Although it is accepted that motor neuron loss is a primary event in disease pathogenesis, inherent defects in muscle may also contribute to the disease progression and severity. In this review, we discuss the relative contributions of primary pathological processes in the motor axons, neuromuscular junctions and muscle to disease manifestations. Characterizing these contributions helps us to better understand the disease mechanisms and to better target therapeutic intervention.

• 出版日期2012