A critical smn threshold in mice dictates onset of an intermediate spinal muscular atrophy phenotype associated with a distinct neuromuscular junction pathology

Neuromuscular Disorders

Volumn 22, Issue 3, 2012, Pages 263-276

  Bowerman, Mélissa ^a,b   Murray, Lyndsay M ^a   Beauvais, Ariane ^a   Pinheiro, Bruno ^a   Kothary, Rashmi ^a,b  

^a OTTAWA HOSPITAL RESEARCH INSTITUTE   (Canada)

^b UNIVERSITY OF OTTAWA   (Canada)

Author keywords

Motor neuron; Mouse model; Neuromuscular junction; Selective vulnerability; Smn threshold; Spinal muscular atrophy

Indexed keywords

SURVIVAL MOTOR NEURON PROTEIN;

ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; CONTROLLED STUDY; DISEASE SEVERITY; GENE; LIFE EXPECTANCY; MOTONEURON; MOTOR DYSFUNCTION; MOUSE; NERVE DEGENERATION; NEUROMUSCULAR JUNCTION DISORDER; NEUROPATHOLOGY; NONHUMAN; PHENOTYPE; PRIORITY JOURNAL; SMN GENE; SPINAL MUSCULAR ATROPHY; WEIGHT REDUCTION;

AGE FACTORS; ANIMALS; ANIMALS, NEWBORN; AXONS; BODY WEIGHT; BRAIN; BUNGAROTOXINS; DISEASE MODELS, ANIMAL; GAIT DISORDERS, NEUROLOGIC; GENE EXPRESSION REGULATION; MICE; MICE, INBRED C57BL; MICE, KNOCKOUT; MOTOR ENDPLATE; MUSCLE, SKELETAL; MUSCULAR ATROPHY, SPINAL; NERVE DEGENERATION; NEUROMUSCULAR DISEASES; NEUROMUSCULAR JUNCTION; PHENOTYPE; REACTION TIME; RECEPTORS, CHOLINERGIC; RNA, MESSENGER; SENSATION DISORDERS; SPINAL CORD; SURVIVAL OF MOTOR NEURON 1 PROTEIN;

EID: 84858072544     PISSN: 09608966     EISSN: 18732364     Source Type: Journal    
DOI: 10.1016/j.nmd.2011.09.007     Document Type: Article

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