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Spinal muscular atrophy, John Griffin, and mentorship
Author(s) -
Sumner Charlotte J.
Publication year - 2012
Publication title -
journal of the peripheral nervous system
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1
H-Index - 67
eISSN - 1529-8027
pISSN - 1085-9489
DOI - 10.1111/j.1529-8027.2012.00432.x
Subject(s) - motor neuron , spinal muscular atrophy , neuroscience , medicine , atrophy , synapse , pathological , disease , neuromuscular junction , weakness , muscle atrophy , griffin , neuron , pathology , anatomy , biology , history , archaeology
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.