title: Spinal muscular atrophy, John Griffin, and mentorship.
abstract: 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.
authors.affiliation: Department of Neurology; Department of Neuroscience, Johns Hopkins School of Medicine, 855 North Wolfe St., Baltimore, MD 21205, USA. csumner1@jhmi.edu
authors.forename: Charlotte J
authors.initials: CJ
authors.lastname: Sumner
mesh_terms: D000818:Animals; D004283:Dog Diseases / Q000473:pathology* / Q000503:physiopathology; D004285:Dogs; D006801:Humans; D009046:Motor Neurons / Q000473:pathology; D009134:Muscular Atrophy, Spinal / Q000473:pathology* / Q000503:physiopathology / Q000662:veterinary*; D009469:Neuromuscular Junction / Q000473:pathology / Q000503:physiopathology