Spinal muscular atrophy, John Griffin, and mentorship

J Peripher Nerv Syst. 2012 Dec:17 Suppl 3:52-6. doi: 10.1111/j.1529-8027.2012.00432.x.


Hereditary canine spinal muscular atrophy is an inherited motor neuron disease that occurs in Brittany Spaniels and has remarkable similarities with human spinal muscular atrophy. Both disorders are characterized by proximal limb and truncal muscle weakness of variable severity. Detailed pathological studies indicate that there is early dysfunction of motor neuron synapses, particularly the neuromuscular junction synapse, prior to motor neuron death. This period of synaptic dysfunction may define a critical window of opportunity for disease reversibility in motor neuron disease.

Publication types

  • Review

MeSH terms

  • Animals
  • Dog Diseases / pathology*
  • Dog Diseases / physiopathology
  • Dogs
  • Humans
  • Motor Neurons / pathology
  • Muscular Atrophy, Spinal / pathology*
  • Muscular Atrophy, Spinal / physiopathology
  • Muscular Atrophy, Spinal / veterinary*
  • Neuromuscular Junction / pathology
  • Neuromuscular Junction / physiopathology