Electrophysiological studies in a mouse model of Schwartz-Jampel syndrome demonstrate muscle fiber hyperactivity of peripheral nerve origin

Muscle and Nerve

Volumn 40, Issue 1, 2009, Pages 55-61

  Echaniz Laguna, Andoni ^a,b,c   Rene, Frédérique ^b,c   Marcel, Christophe ^a   Bangratz, Marie ^b,d   Fontaine, Bertrand ^b,d,e   Loeffler, Jean Philippe ^b,c   Nicole, Sophie ^b,d,e  

^a HÔPITAL CIVIL   (France)

^b INSERM   (France)

^c UNIVERSITÉ DE STRASBOURG   (France)

^d SORBONNE UNIVERSITÉ   (France)

^e ASSISTANCE PUBLIQUE HÔPITAUX DE PARIS   (France)

Author keywords

Mouse model; Neuromyotonia; Peripheral nerve hyperexcitability; Perlecan; Schwartz Jampel syndrome

Indexed keywords

PERLECAN;

ANIMAL EXPERIMENT; ANIMAL MODEL; ARTICLE; AUTOSOMAL RECESSIVE DISORDER; CHONDRODYSPLASIA; CONTROLLED STUDY; ELECTROMYOGRAM; FEMALE; GENERAL ANESTHESIA; MALE; MISSENSE MUTATION; MOTOR UNIT POTENTIAL; MOUSE; MUSCLE STIFFNESS; NERVE DEGENERATION; NERVE STIMULATION; NONHUMAN; PERIPHERAL NERVE; PRIORITY JOURNAL; SCHWARTZ JAMPEL SYNDROME; SENSORY NERVE CONDUCTION;

ACTION POTENTIALS; ANIMALS; BIOPHYSICAL PHENOMENA; CURARE; DISEASE MODELS, ANIMAL; ELECTRIC STIMULATION; ELECTROMYOGRAPHY; HEPARAN SULFATE PROTEOGLYCANS; MICE; MICE, TRANSGENIC; MUSCLE FIBERS, SKELETAL; MUTATION, MISSENSE; NEURAL CONDUCTION; NEUROMUSCULAR NONDEPOLARIZING AGENTS; OSTEOCHONDRODYSPLASIAS; PERIPHERAL NERVES; REACTION TIME;

EID: 67650230924     PISSN: 0148639X     EISSN: 10974598     Source Type: Journal    
DOI: 10.1002/mus.21253     Document Type: Article

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