Ultrastructural changes in diaphragm neuromuscular junctions in a severe mouse model for Spinal Muscular Atrophy and their prevention by bifunctional U7 snRNA correcting SMN2 splicing

Neuromuscular Disorders

Volumn 20, Issue 11, 2010, Pages 744-752

  Voigt, Tilman ^b   Meyer, Kathrin ^a   Baum, Oliver ^b   Schümperli, Daniel ^a  

^a UNIVERSITY OF BERN   (Switzerland)

^b UNIVERSITY OF BERN   (Switzerland)

Author keywords

Diaphragm; Mouse models; Neuromuscular junctions; Perisynaptic Schwann cells; Spinal muscular atrophy

Indexed keywords

SMALL NUCLEAR RIBONUCLEOPROTEIN; SMALL NUCLEAR RNA; SURVIVAL MOTOR NEURON PROTEIN; SURVIVAL MOTOR NEURON PROTEIN 1; SURVIVAL MOTOR NEURON PROTEIN 2;

ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; DIAPHRAGM; DISEASE SEVERITY; ENDONEURIUM; FEMALE; FIBROBLAST; GENE DELETION; GENE INACTIVATION; MALE; MITOCHONDRION; MOUSE; MUSCLE FUNCTION; NERVE CELL NECROSIS; NEUROMUSCULAR SYNAPSE; NONHUMAN; PATHOGENESIS; PRESYNAPTIC NERVE; PRIORITY JOURNAL; RNA SPLICING; SCHWANN CELL; SKELETAL MUSCLE; SOLEUS MUSCLE; SPINAL CORD MOTONEURON; SPINAL MUSCULAR ATROPHY; ULTRASTRUCTURE;

ANIMALS; DIAPHRAGM; DISEASE MODELS, ANIMAL; MICE; MICE, TRANSGENIC; MICROSCOPY, ELECTRON, TRANSMISSION; MITOCHONDRIA; MOTOR NEURONS; MUSCLE, SKELETAL; MUSCULAR ATROPHY, SPINAL; NEUROMUSCULAR JUNCTION; REVERSE TRANSCRIPTASE POLYMERASE CHAIN REACTION; SURVIVAL OF MOTOR NEURON 2 PROTEIN;

EID: 77957322783     PISSN: 09608966     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.nmd.2010.06.010     Document Type: Article

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